2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM …10.3.1. Recommendations for Counseling and Management of Chronic Aortic Diseases in Pregnancy.....e89 10.4. Evaluation and Management

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Journal of the American College of Cardiology Vol. 55, No. 14, 2010© 2010 by the American College of Cardiology Foundation and the American Heart Association, Inc. ISSN 0735-1097/10/$36.00P

PRACTICE GUIDELINE: FULL TEXT

2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVMGuidelines for the Diagnosis and Management ofPatients With Thoracic Aortic DiseaseA Report of the American College of Cardiology Foundation/American Heart Association Task Forceon Practice Guidelines, American Association for Thoracic Surgery, American College of Radiology,American Stroke Association, Society of Cardiovascular Anesthesiologists, Society for CardiovascularAngiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons,and Society for Vascular Medicine

Endorsed by the North American Society for Cardiovascular Imaging

WRITING GROUP MEMBERSLoren F. Hiratzka, MD, Chair*; George L. Bakris, MD†; Joshua A. Beckman, MD, MS‡;Robert M. Bersin, MD§; Vincent F. Carr, DO�; Donald E. Casey Jr, MD, MPH, MBA¶;

Kim A. Eagle, MD*#; Luke K. Hermann, MD**; Eric M. Isselbacher, MD*;Ella A. Kazerooni, MD, MS††; Nicholas T. Kouchoukos, MD‡‡; Bruce W. Lytle, MD§§;

Dianna M. Milewicz, MD, PhD; David L. Reich, MD��; Souvik Sen, MD, MS¶¶;Julie A. Shinn, RN, MA, CCRN†; Lars G. Svensson, MD, PhD##;

David M. Williams, MD#***

ACCF/AHA TASK FORCE MEMBERSAlice K. Jacobs, MD, FACC, FAHA, Chair 2009–2011; Sidney C. Smith, Jr, MD, FACC, FAHA,

Immediate Past Chair 2006–2008†††; Jeffery L. Anderson, MD, FACC, FAHA, Chair-Elect;Cynthia D. Adams, MSN, PhD, FAHA†††; Christopher E. Buller, MD, FACC;

Mark A. Creager, MD, FACC, FAHA; Steven M. Ettinger, MD, FACC;Robert A. Guyton, MD, FACC, FAHA; Jonathan L. Halperin, MD, FACC, FAHA;

Sharon A. Hunt, MD, FACC, FAHA†††; Harlan M. Krumholz, MD, FACC, FAHA†††;Frederick G. Kushner, MD, FACC, FAHA; Bruce W. Lytle, MD, FACC, FAHA†††;Rick Nishimura, MD, FACC, FAHA†††; Richard L. Page, MD, FACC, FAHA†††;Barbara Riegel, DNSc, RN, FAHA***; William G. Stevenson, MD, FACC, FAHA;

Lynn G. Tarkington, RN; Clyde W. Yancy, MD, FACC, FAHA

ACCF/AHA Representative. †AHA Representative. ‡SVM Representative. §SCAI Representative. �ACCF Board of Governors Representative.American College of Physicians Representative. #Recused from Section 9.2.2.3.1. Recommendations for Descending Thoracic Aorta and Thoracoab-ominal Aortic Aneurysms. **American College of Emergency Physicians Representative. ††ACR Representative. ‡‡STS Representative. §§ACCF/HA Task Force Liaison. ��SCA Representative. ¶¶ASA Representative. ##AATS Representative. ***SIR Representative. †††Former Task Forceember during this writing effort.Authors with no symbol by their name were included to provide additional content expertise apart from organizational representation.This document was approved by the American College of Cardiology Foundation Board of Trustees and the American Heart Association Science

dvisory and Coordinating Committee in January 2010. All other cosponsoring organizations approved in February 2010.The American College of Cardiology Foundation requests that this document be cited as follows: Hiratzka LF, Bakris GL, Beckman JA, Bersin RM,

arr VF, Casey DE Jr, Eagle KA, Hermann LK, Isselbacher EM, Kazerooni EA, Kouchoukos NT, Lytle BW, Milewicz DM, Reich DL, Sen S, ShinnA, Svensson LG, Williams DM. 2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVM Guidelines for the diagnosis and management ofatients with thoracic aortic disease: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practiceuidelines, American Association for Thoracic Surgery, American College of Radiology, American Stroke Association, Society of Cardiovascularnesthesiologists, Society for Cardiovascular Angiography and Interventions, Society of Interventional Radiology, Society of Thoracic Surgeons, andociety for Vascular Medicine (developed in collaboration with the American College of Emergency Physicians). J Am Coll Cardiol 2010;55:e27–129.This article has been copublished in Circulation.Copies: This document is available on the World Wide Web sites of the American College of Cardiology (www.acc.org) and the American Heart

ssociation (my.americanheart.org). For copies of this document, please contact Elsevier Inc. Reprint Department, fax 212-633-3820, e-mail:[email protected].

ublished by Elsevier Inc. doi:10.1016/j.jacc.2010.02.015

Permissions: Multiple copies, modification, alteration, enhancement, and/or distribution of this document are not permitted without the expressermission of the American College of Cardiology Foundation. Please contact Elsevier’s permission department at [email protected].

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e28 Hiratzka et al. JACC Vol. 55, No. 14, 20102010 Guidelines on Thoracic Aortic Disease April 6, 2010:e27–129

TABLE OF CONTENTS

reamble . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .e31

1. Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .e31

1.1. Methodology and Evidence Review . . . . . .e311.2. Organization of the Writing Committee . .e321.3. Document Review and Approval . . . . . . . . .e321.4. Scope of the Guideline . . . . . . . . . . . . . . . . . .e33

1.4.1. Critical Issues . . . . . . . . . . . . . . . . . . . . .e351.5. Glossary of Terms and Abbreviations Used

Throughout Guideline . . . . . . . . . . . . . . . . . . .e35

2. The Thoracic Aorta . . . . . . . . . . . . . . . . . . . . . . . . . . .e36

2.1. The Normal Aorta . . . . . . . . . . . . . . . . . . . . . . .e362.2. Normal Thoracic Aortic Diameter . . . . . . . .e36

3. Thoracic Aortic Histopathology . . . . . . . . . . . . . . .e37

3.1. Atherosclerosis . . . . . . . . . . . . . . . . . . . . . . . . .e373.2. Aneurysms and Dissections . . . . . . . . . . . . .e373.3. Vasculitis and Inflammatory Diseases. . . .e38

4. Imaging Modalities . . . . . . . . . . . . . . . . . . . . . . . . . . .e39

4.1. Recommendations for Aortic ImagingTechniques to Determine the Presence andProgression of Thoracic Aortic Disease . .e39

4.2. Chest X-Ray . . . . . . . . . . . . . . . . . . . . . . . . . . . .e404.3. Computed Tomographic Imaging . . . . . . . . .e40

4.3.1. Computed Tomographic ImagingTechnique . . . . . . . . . . . . . . . . . . . . . . . .e42

4.4. Magnetic Resonance Imaging . . . . . . . . . . .e424.4.1. Magnetic Resonance Imaging

Technique . . . . . . . . . . . . . . . . . . . . . . . .e434.4.2. Black Blood Imaging . . . . . . . . . . . . . . .e434.4.3. Noncontrast White Blood Imaging . . .e434.4.4. Contrast-Enhanced Magnetic Resonance

Angiography. . . . . . . . . . . . . . . . . . . . . . .e434.4.5. Phase Contrast Imaging. . . . . . . . . . . . .e43

4.5. Standards for Reporting of the ThoracicAorta on Computed Tomography andMagnetic Resonance Imaging . . . . . . . . . . .e43

4.6. Angiography . . . . . . . . . . . . . . . . . . . . . . . . . . . .e444.7. Echocardiography . . . . . . . . . . . . . . . . . . . . . . .e45

4.7.1. Echocardiographic Criteria for ThoracicAortic Aneurysms . . . . . . . . . . . . . . . . . .e45

4.7.2. Echocardiographic Criteria for AorticDissection. . . . . . . . . . . . . . . . . . . . . . . . .e454.7.2.1. DIAGNOSTIC ACCURACY OF

ECHOCARDIOGRAPHY FOR AORTICDISSECTION. . . . . . . . . . . . . . . . . . . . . . .e46

4.7.2.2. DIAGNOSTIC ACCURACY OFECHOCARDIOGRAPHY FOR ACUTEINTRAMURAL HEMATOMA . . . . . . .e46

4.7.2.3. ROLE OF ECHOCARDIOGRAPHY INFOLLOWING PATIENTS WITHCHRONIC AORTIC DISEASE . . . . . .e47

5. Genetic Syndromes Associated With ThoracicAortic Aneurysms and Dissections . . . . . . . . . . . .e47

5.1. Recommendations for GeneticSyndromes . . . . . . . . . . . . . . . . . . . . . . . . . . . . .e475.1.1. Marfan Syndrome . . . . . . . . . . . . . . . . . .e48
5.1.2. Loeys-Dietz Syndrome . . . . . . . . . . . . . .e49
5.1.3. Ehlers-Danlos Syndrome, Vascular Formor Type IV . . . . . . . . . . . . . . . . . . . . . . . .e49

5.1.4. Turner Syndrome . . . . . . . . . . . . . . . . . .e495.1.5. Other Genetic Syndromes With

Increased Risk for Thoracic AorticAneurysms and Dissections . . . . . . . . . .e50

5.1.6. Recommendations for Familial ThoracicAortic Aneurysms and Dissections . . . .e50

5.2. Summary . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .e51

6. Other Cardiovascular Conditions Associated WithThoracic Aortic Aneurysm and Dissection. . . . . .e52

6.1. Recommendations for Bicuspid Aortic Valveand Associated Congenital Variants inAdults . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .e52

6.2. Aberrant Right Subclavian Artery . . . . . . . .e536.3. Coarctation of the Aorta . . . . . . . . . . . . . . . .e536.4. Right Aortic Arch . . . . . . . . . . . . . . . . . . . . . . .e53

7. Inflammatory Diseases Associated With ThoracicAortic Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .e53

7.1. Recommendations for Takayasu Arteritisand Giant Cell Arteritis . . . . . . . . . . . . . . . . . .e53

7.2. Takayasu Arteritis . . . . . . . . . . . . . . . . . . . . . .e547.3. Giant Cell Arteritis . . . . . . . . . . . . . . . . . . . . . .e567.4. Behcet Disease . . . . . . . . . . . . . . . . . . . . . . . . .e577.5. Ankylosing Spondylitis

(Spondyloarthropathies) . . . . . . . . . . . . . . . .e577.6. Infective Thoracic Aortic Aneurysms . . . . .e57

8. Acute Aortic Syndromes . . . . . . . . . . . . . . . . . . . . . .e58

8.1. Aortic Dissection . . . . . . . . . . . . . . . . . . . . . . .e588.1.1. Aortic Dissection Definition . . . . . . . .e588.1.2. Anatomic Classification of Aortic

Dissection. . . . . . . . . . . . . . . . . . . . . . . .e588.1.3. Risk Factors for Aortic Dissection . . .e618.1.4. Clinical Presentation of Acute Thoracic

Aortic Dissection . . . . . . . . . . . . . . . . .e628.1.4.1. SYMPTOMS OF ACUTE THORACIC

AORTIC DISSECTION . . . . . . . . . . . .e628.1.4.2. PERFUSION DEFICITS AND END-

ORGAN ISCHEMIA . . . . . . . . . . . . . . .e628.1.5. Cardiac Complications . . . . . . . . . . . . .e64

8.1.5.1. ACUTE AORTICREGURGITATION . . . . . . . . . . . . . . . .e64

8.1.5.2. MYOCARDIAL ISCHEMIA ORINFARCTION . . . . . . . . . . . . . . . . . . . . .e64

8.1.5.3. HEART FAILURE AND SHOCK . . .e648.1.5.4. PERICARDIAL EFFUSION AND

TAMPONADE. . . . . . . . . . . . . . . . . . . . .e648.1.6. Syncope . . . . . . . . . . . . . . . . . . . . . . . . .e648.1.7. Neurologic Complications . . . . . . . . . .e658.1.8. Pulmonary Complications . . . . . . . . . .e658.1.9. Gastrointestinal Complications. . . . . .e65

8.1.10. Blood Pressure and Heart RateConsiderations . . . . . . . . . . . . . . . . . . . .e65

8.1.11. Age and Sex Considerations . . . . . . . .e658.2. Intramural Hematoma . . . . . . . . . . . . . . . . . . .e668.3. Penetrating Atherosclerotic Ulcer . . . . . . .e678.4. Pseudoaneurysms of the Thoracic Aorta .e678.5. Traumatic Rupture of the Thoracic Aorta .e678.6. Evaluation and Management of Acute

Thoracic Aortic Disease . . . . . . . . . . . . . . . . .e68

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8.6.1. Initial Evaluation and Management. . .e688.6.1.1. RECOMMENDATIONS FOR

ESTIMATION OF PRETEST RISK OFTHORACIC AORTIC DISSECTION . .e68

8.6.1.2. LABORATORY TESTING. . . . . . . . . . .e688.6.1.3. RECOMMENDATIONS FOR

SCREENING TESTS . . . . . . . . . . . . . . . .e698.6.1.4. RECOMMENDATIONS FOR

DIAGNOSTIC IMAGING STUDIES . .e698.6.1.5. RECOMMENDATIONS FOR INITIAL

MANAGEMENT . . . . . . . . . . . . . . . . . . . .e698.6.1.6. RECOMMENDATIONS FOR

DEFINITIVE MANAGEMENT . . . . . .e698.6.2. Evaluation and Management

Algorithms . . . . . . . . . . . . . . . . . . . . . . . .e708.6.3. Initial Management . . . . . . . . . . . . . . . .e71

8.6.3.1. BLOOD PRESSURE AND RATECONTROL THERAPY . . . . . . . . . . . . . .e71

8.6.3.2. ADDITIONAL ANTIHYPERTENSIVETHERAPY . . . . . . . . . . . . . . . . . . . . . . . . . .e73

8.6.3.3. PAIN CONTROL . . . . . . . . . . . . . . . . . . .e738.6.3.4. HYPOTENSION . . . . . . . . . . . . . . . . . . . .e738.6.3.5. DETERMINING DEFINITIVE

MANAGEMENT . . . . . . . . . . . . . . . . . . . .e738.6.4. Recommendation for Surgical

Intervention for Acute Thoracic AorticDissection. . . . . . . . . . . . . . . . . . . . . . . . .e73

8.6.5. Endovascular Interventions . . . . . . . . . .e738.6.6. Principles of Treatment for Intramural

Hematoma and PenetratingAtherosclerotic Ulcer . . . . . . . . . . . . . . .e748.6.6.1. INTIMAL DEFECT WITHOUT

INTRAMURAL HEMATOMA . . . . . . .e748.6.6.2. INTIMAL DEFECT WITH

INTRAMURAL HEMATOMA . . . . . . .e748.6.6.3. RECOMMENDATION FOR

INTRAMURAL HEMATOMAWITHOUT INTIMAL DEFECT . . . . .e74

8.7. Treatment for the Management ofTraumatic Aortic Rupture. . . . . . . . . . . . . . . .e74

9. Thoracic Aortic Aneurysms . . . . . . . . . . . . . . . . . . .e75

9.1. General Approach to the Patient. . . . . . . . .e769.1.1. Recommendation for History and

Physical Examination for ThoracicAortic Disease . . . . . . . . . . . . . . . . . . . . .e769.1.1.1. CORONARY ARTERY DISEASE . . . .e779.1.1.2. EMBOLI . . . . . . . . . . . . . . . . . . . . . . . . . . .e789.1.1.3. ASSOCIATED RENAL ISCHEMIA . .e789.1.1.4. ASSOCIATED MESENTERIC

ISCHEMIA . . . . . . . . . . . . . . . . . . . . . . . . .e789.1.1.5. ASSOCIATED PERIPHERAL

ISCHEMIA . . . . . . . . . . . . . . . . . . . . . . . . .e789.1.2. Differential Diagnosis. . . . . . . . . . . . . . .e78

9.1.2.1. SYMPTOMS . . . . . . . . . . . . . . . . . . . . . . . .e789.1.2.2. PHYSICAL FINDINGS . . . . . . . . . . . . . .e78

9.1.3. Considerations for Imaging . . . . . . . . . .e799.2. General Medical Treatment and Risk Factor

Management for Patients With ThoracicAortic Disease . . . . . . . . . . . . . . . . . . . . . . . . . .e799.2.1. Recommendation for Medical Treatment

of Patients With Thoracic AorticDiseases . . . . . . . . . . . . . . . . . . . . . . . . . .e799.2.1.1. RECOMMENDATIONS FOR BLOOD

PRESSURE CONTROL . . . . . . . . . . . . .e80

9.2.1.2. RECOMMENDATION FORDYSLIPIDEMIA . . . . . . . . . . . . . . . . . . . .e80

9.2.1.3. RECOMMENDATION FOR SMOKINGCESSATION. . . . . . . . . . . . . . . . . . . . . . . .e80

9.2.2. Surgical and Endovascular Treatment byLocation of Disease . . . . . . . . . . . . . . . .e819.2.2.1. ASCENDING AORTA AND AORTIC

SINUSES . . . . . . . . . . . . . . . . . . . . . . . . . . .e819.2.2.1.1. Recommendations for

Asymptomatic PatientsWith AscendingAortic Aneurysm . . . . . .e81

9.2.2.1.2. Recommendation forSymptomatic PatientsWith ThoracicAortic Aneurysm . . . . . .e81

9.2.2.1.3. Endovascular Graftingfor Ascending AorticAneurysm. . . . . . . . . . . .e82

9.2.2.1.4. Recommendations forOpen Surgery forAscending AorticAneurysm. . . . . . . . . . . .e82

9.2.2.2. RECOMMENDATIONS FOR AORTICARCH ANEURYSMS . . . . . . . . . . . . . . . .e849.2.2.2.1. Open Surgery . . . . . . . . .e84

9.2.2.3. DESCENDING THORACIC AORTAAND THORACOABDOMINALAORTA . . . . . . . . . . . . . . . . . . . . . . . . . . . .e849.2.2.3.1. Recommendations for

Descending ThoracicAorta andThoracoabdominalAortic Aneurysms . . . . .e84

9.2.2.3.2. Endovascular VersusOpen SurgicalApproach . . . . . . . . . . . .e85

9.2.2.3.3. End-OrganPreservationDuring ThoracicEndograftImplantation. . . . . . . . . .e86

9.2.2.3.4. PeriproceduralComplications ofEndograftProcedures . . . . . . . . . . .e87

9.2.2.3.5. Open Surgical . . . . . . . .e889.2.2.3.6. End-Organ

PreservationDuring OpenThoracoabdominalRepairs . . . . . . . . . . . . . .e89

9.2.2.3.7. Aortic Dissection WithMalperfusion . . . . . . . . .e89

0. Special Considerations in Pregnant Patients WithAortic Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .e89

10.1. Effects of Pregnancy on the Aorta. . . . . .e8910.2. Epidemiology of Chronic and Acute Aortic

Conditions in Pregnancy . . . . . . . . . . . . . . .e8910.3. Counseling and Management of Chronic

Aortic Diseases in Pregnancy . . . . . . . . . .e90

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10.3.1. Recommendations for Counseling andManagement of Chronic AorticDiseases in Pregnancy . . . . . . . . . . . .e89

10.4. Evaluation and Management of AcuteAortic Syndromes During Pregnancy . . . .e90

1. Aortic Arch and Thoracic Aortic Atheroma andAtheroembolic Disease . . . . . . . . . . . . . . . . . . . . . . .e90

11.1. Recommendations for Aortic Arch andThoracic Aortic Atheroma andAtheroembolic Disease . . . . . . . . . . . . . . . .e90

11.2. Clinical Description . . . . . . . . . . . . . . . . . . . .e9011.3. Risk Factors. . . . . . . . . . . . . . . . . . . . . . . . . . .e9111.4. Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . .e9111.5. Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . .e91

11.5.1. Anticoagulation Versus AntiplateletTherapy . . . . . . . . . . . . . . . . . . . . . . . .e91

11.5.2. Lipid-Lowering Agent . . . . . . . . . . . .e9211.5.3. Surgical and Interventional

Approaches . . . . . . . . . . . . . . . . . . . . .e92

2. Porcelain Aorta . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .e92

3. Tumors of the Thoracic Aorta . . . . . . . . . . . . . . . . .e93

4. Perioperative Care for Open Surgical andEndovascular Thoracic Aortic Repairs . . . . . . . . .e93

14.1. Recommendations for PreoperativeEvaluation. . . . . . . . . . . . . . . . . . . . . . . . . . . . .e9314.1.1. Preoperative Risk Assessment. . . . . .e94

14.2. Recommendations for Choice ofAnesthetic and Monitoring Techniques. .e9514.2.1. Temperature Monitoring . . . . . . . . . .e9514.2.2. Hemodynamic Monitoring . . . . . . . .e9514.2.3. Transesophageal Echocardiography . .e9614.2.4. Transesophageal Echocardiography for

Endovascular Repairs of theDescending Thoracic Aorta. . . . . . . .e96

14.3. Airway Management for DescendingThoracic Aortic Repairs . . . . . . . . . . . . . . . .e96

14.4. Recommendation for TransfusionManagement and Anticoagulation inThoracic Aortic Surgery . . . . . . . . . . . . . . . .e96

14.5. Organ Protection . . . . . . . . . . . . . . . . . . . . . .e9714.5.1. Recommendations for Brain Protection

During Ascending Aortic andTransverse Aortic Arch Surgery . . . .e97

14.5.2. Recommendations for Spinal CordProtection During Descending AorticOpen Surgical and EndovascularRepairs . . . . . . . . . . . . . . . . . . . . . . . . .e9814.5.2.1. MONITORING OF SPINAL

CORD FUNCTION INDESCENDING THORACICAORTIC REPAIRS. . . . . . . . . . . . . .e99

14.5.2.2. MAINTENANCE OF SPINAL CORDARTERIAL PRESSURE. . . . . . . . . .e99

14.5.2.3. CEREBROSPINAL FLUIDPRESSURE AND DRAINAGE . . .e99

14.5.2.4. HYPOTHERMIA . . . . . . . . . . . . . .e10014.5.2.5. GLUCOCORTICOIDS AND

MANNITOL . . . . . . . . . . . . . . . . . . .e10014.5.3. Recommendations for Renal

Protection During Descending Aortic

Open Surgical and EndovascularRepairs . . . . . . . . . . . . . . . . . . . . . . . .e100

14.6. Complications of Open SurgicalApproaches . . . . . . . . . . . . . . . . . . . . . . . . . .e100

14.7. Mortality Risk for Thoracic AorticSurgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .e101

14.8. Postprocedural Care . . . . . . . . . . . . . . . . . .e10214.8.1. Postoperative Risk Factor

Management . . . . . . . . . . . . . . . . . . .e10214.8.2. Recommendations for Surveillance of

Thoracic Aortic Disease or PreviouslyRepaired Patients . . . . . . . . . . . . . . .e102

5. Nursing Care and Patient/Family Education . .e103

15.1. Nursing Care of Medically ManagedPatients. . . . . . . . . . . . . . . . . . . . . . . . . . . . . .e103

15.2. Preprocedural Nursing Care . . . . . . . . . . .e10315.3. Postprocedural Nursing Care . . . . . . . . . .e10315.4. Nursing Care of Surgically Managed

Patients. . . . . . . . . . . . . . . . . . . . . . . . . . . . . .e104

6. Long-Term Issues . . . . . . . . . . . . . . . . . . . . . . . . . . .e105

16.1. Recommendation for Employment andLifestyle in Patients With Thoracic AorticDisease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .e105

7. Institutional/Hospital Quality Concerns . . . . . .e106

17.1. Recommendations for Quality Assessmentand Improvement for Thoracic AorticDisease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .e106

17.2. Interinstitutional Issues . . . . . . . . . . . . . .e107

8. Future Research Directions and Issues. . . . . . .e107

18.1. Risks and Benefits of Current ImagingTechnologies . . . . . . . . . . . . . . . . . . . . . . . . .e107

18.2. Mechanisms of Aortic Dissection . . . . . .e10818.3. Treatment of Malperfusion and

Reperfusion Injury . . . . . . . . . . . . . . . . . . . .e10818.4. Gene-Based Mechanisms and Models . .e108

18.4.1. Aortic Disease Management Based onthe Underlying Genetic Defects . . .e108

18.4.2. Biomarkers for Acute AorticDissection . . . . . . . . . . . . . . . . . . . . .e108

18.4.3. Genetic Defects and MolecularPathway Analyses . . . . . . . . . . . . . . .e108

18.4.4. Clinical Trials for Medical Therapy forAortic Aneurysms . . . . . . . . . . . . . . .e108

18.5. Aortic Atheroma and AtherosclerosisIdentification and Treatment . . . . . . . . . .e108

18.6. Prediction Models of Aortic Rupture and theNeed for Preemptive Interventions . . . . .e108

18.7. National Heart, Lung, and Blood InstituteWorking Group Recommendations . . . . .e108

eferences . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .e109

ppendix 1. Author Relationships With Industry andther Entities . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .e126

ppendix 2. Reviewer Relationships With Industryd Other Entities. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .e128

ppendix 3. Abbreviation List . . . . . . . . . . . . . . . . . . . .e130

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is essential that the medical profession play a central role initically evaluating the evidence related to drugs, devices,d procedures for the detection, management, or preventiondisease. Properly applied, rigorous, expert analysis of theailable data documenting absolute and relative benefits and

sks of these therapies and procedures can improve outcomesd reduce costs of care by focusing resources on the most

fective strategies. One important use of such data is theoduction of clinical practice guidelines that, in turn, canovide a foundation for a variety of other applications suchperformance measures, appropriate use criteria, clinical

cision support tools, and quality improvement tools.The American College of Cardiology Foundation (ACCF)d the American Heart Association (AHA) have jointlygaged in the production of guidelines in the area ofrdiovascular disease since 1980. The ACCF/AHA Taskrce on Practice Guidelines is charged with developing,dating, and revising practice guidelines for cardiovascularseases and procedures, and the Task Force directs andersees this effort. Writing committees are charged withsessing the evidence as an independent group of authors tovelop, update, or revise recommendations for clinicalactice.Experts in the subject under consideration have beenlected from both organizations to examine subject-specificta and write guidelines in partnership with representatives

om other medical practitioner and specialty groups. Writingmmittees are specifically charged to perform a formalerature review, weigh the strength of evidence for orainst particular treatments or procedures, and includetimates of expected health outcomes where data exist.tient-specific modifiers, comorbidities, and issues of pa-nt preference that may influence the choice of tests orerapies are considered. When available, information fromudies on cost is considered, but data on efficacy and clinicaltcomes constitute the primary basis for recommendationsthese guidelines.The ACCF/AHA Task Force on Practice Guidelines makesery effort to avoid actual, potential, or perceived conflictsinterest that may arise as a result of industry relationshipspersonal interests among the writing committee. Specifi-

lly, all members of the writing committee, as well as peerviewers of the document, are asked to disclose all currentlationships and those 24 months prior to initiation of theriting effort that may be perceived as relevant. All guidelinecommendations require a confidential vote by the writingmmittee and must be approved by a consensus of theembers voting. Members who were recused from voting areted on the title page of this document. Members mustcuse themselves from voting on any recommendationhere their relationships with industry (RWI) apply. If ariting committee member develops a new relationship withdustry during his/her tenure, he/she is required to notifyideline staff in writing. These statements are reviewed bye Task Force on Practice Guidelines and all membersring each conference call and/or meeting of the writing
mmittee, updated as changes occur, and ultimately pub- re
hed as an appendix to the document. For detailed informa-n regarding guideline policies and procedures, please referthe methodology manual for ACCF/AHA Guideline Writ-

g Committees (1). RWI and other entities pertinent to thisideline for authors and peer reviewers are disclosed in

ppendixes 1 and 2, respectively. Disclosure information fore ACCF/AHA Task Force on Practice Guidelines is alsoailable online at (http://www.acc.org/about/overview/

linicalDocumentsTaskForces.cfm).These practice guidelines are intended to assist healthcareoviders in clinical decision making by describing a range ofnerally acceptable approaches for diagnosis, management,d prevention of specific diseases or conditions. Cliniciansould consider the quality and availability of expertise in theea where care is provided. These guidelines attempt tofine practices that meet the needs of most patients in mostrcumstances. The recommendations reflect a consensuster a thorough review of the available current scientificidence and are intended to improve patient care. The Taskrce recognizes that situations arise where additional data

e needed to better inform patient care; these areas will beentified within each respective guideline when appropriate.Patient adherence to prescribed and agreed upon medicalgimens and lifestyles is an important aspect of treatment.escribed courses of treatment in accordance with thesecommendations are effective only if they are followed.ecause lack of patient understanding and adherence mayversely affect outcomes, physicians and other healthcareoviders should make every effort to engage the patient’stive participation in prescribed medical regimens and life-yles.If these guidelines are used as the basis for regulatory oryer decisions, the goal should be improvement in quality ofre and aligned with the patient’s best interest. The ultimatedgment regarding care of a particular patient must be madethe healthcare provider and the patient in light of all of the

rcumstances presented by that patient. Consequently, theree circumstances in which deviations from these guidelinese appropriate.The guidelines will be reviewed annually by the ACCF/

HA Task Force on Practice Guidelines and consideredrrent unless they are updated, revised, or withdrawn fromstribution.

Alice K. Jacobs, MD, FACC, FAHAChair, ACCF/AHA Task Force on Practice Guidelines

Sidney C. Smith, Jr, MD, FACC, FAHAImmediate Past Chair, ACCF/AHA Task Force on

Practice Guidelines

. Introduction

.1. Methodology and Evidence Reviewhe writing committee conducted a comprehensive search ofe medical and scientific literature through the use of-bMed/MEDLINE. Searches were limited to publications

ritten in the English language. Compiled reports were
viewed and additional articles were provided by committee
http://www.acc.org/about/overview/ClinicalDocumentsTaskForces.cfm

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embers. Specifically targeted searches were conducted one following subtopics: acute aortic dissection, ankylosingondylitis, aortic dissection and litigation, aortic neoplasm,rtic tumors, Behçet disease, bicuspid aortic valve, calcifiedrta, chronic dissection, coarctation of the aorta, D-dimer,ssecting aneurysm, Ehlers-Danlos syndrome, endovasculard aortic aneurysms, medial degeneration, porcelain aorta,ant cell arteritis, imaging and thoracic aortic disease,flammatory disease, intramural hematoma, Loeys-Dietzndrome, Marfan syndrome, Noonan syndrome, penetratingrtic ulcer, polycystic kidney disease, thoracic and aorticeurysms, thoracic aortic disease and patient care, thoracicrtic disease and surgery, thoracic aorta and Kawasakisease, Takayasu arteritis, thoracoabdominal and aorta orrtic disease, and Turner syndrome. More than 850 refer-ces were reviewed, with 829 used as the primary evidencese for the final guideline. The ACCF/AHA Task Force onactice Guidelines methodology processes were followed torite the text and recommendations. In general, publishedanuscripts appearing in journals listed in Index Medicusere used as the evidence base. Published abstracts were usedly for emerging information but were not used in thermulation of recommendations.The committee reviewed and ranked evidence supportingrrent recommendations with the weight of evidence rankedLevel A if the data were derived from multiple randomized

inical trials or meta-analyses. The committee ranked avail-le evidence as Level B when data were derived from a

ngle randomized trial or nonrandomized studies. Evidenceas ranked as Level C when the primary source of thecommendation was consensus opinion, case studies, orandard of care. In the narrative portions of these guidelines,idence is generally presented in chronological order ofvelopment. Studies are identified as observational, retro-ective, prospective, or randomized. For certain conditionsr which inadequate data are available, recommendations aresed on expert consensus and clinical experience and arenked as Level C. An analogous example is the use ofnicillin for pneumococcal pneumonia, where there are nondomized trials and treatment is based on clinical experi-ce. When recommendations at Level C are supported bystorical clinical data, appropriate references (includinginical reviews) are cited if available. For issues wherearse data are available, a survey of current practice amonge clinicians on the writing committee formed the basis forevel C recommendations and no references are cited. Thehema for classification of recommendations and level ofidence is summarized in Table 1, which also illustrates howe grading system provides an estimate of the size of theeatment effect and an estimate of the certainty of theeatment effect.To provide clinicians with a comprehensive set of data,

henever possible, the exact event rates in various treatmentms of clinical trials are presented to permit calculation ofe absolute risk difference (ARD), number needed to harmNH); the relative treatment effects are described either asds ratio (OR), relative risk (RR), or hazard ratio (HR)
pending on the format in the original publication. Along SC
ith all other point statistics, confidence intervals (CIs) forose statistics are added when available.The writing committee recognized that the evidence baser this guideline is less robust in terms of randomizedinical trials than prior ACCF/AHA guidelines, particularlyose focused on coronary artery disease (CAD) and heartilure. As the reader will discern, much of the evidence baser this topic consists of cohort studies and retrospectiveviews, which largely emanate from centers with a special-ed interest in specific types of thoracic aortic disease. Theriting committee attempted to focus on providing theactitioner with recommendations for evaluation and treat-ent wherever possible and where controversy exists, iden-ed as such in the text.The writing committee acknowledges the expertise of theghly experienced and effective practice guidelines staff ofe ACCF and AHA. The writing committee chair alsoknowledges the commitment and dedication of the diverseriting committee members who were able to put aside issues

specialty “turf” and focus on providing the medicalmmunity with a guideline aimed at optimal patient care.

.2. Organization of the Writing Committeehe guideline was written by a committee comprised ofperts in cardiovascular medicine, surgery, radiology, andrsing. For many of the previous ACCF/AHA practiceidelines, writing expertise has been available within these 2ganizations. Because of the broad scope and diversity oforacic aortic diseases, as well as the specialists who treatch patients, the ACCF and AHA sought greater involve-ent from many specialty organizations. Most, but not all,ecialty organizations that represent the major stakeholdersring for patients with thoracic aortic diseases providedriting committee members and financial support of theoject, and they are recognized as marquee level partnersith the ACCF and AHA. These organizations included themerican Association for Thoracic Surgery (AATS), Amer-an College of Radiology (ACR), American Stroke Associ-ion (ASA), Society of Cardiovascular AnesthesiologistsCA), Society for Cardiovascular Angiography and Inter-ntions (SCAI), Society of Interventional Radiology (SIR),ciety of Thoracic Surgeons (STS), and Society for Vascu-

r Medicine (SVM). The American College of Emergencyysicians (ACEP) and the American College of PhysiciansCP) were also represented on the writing committee.here additional expertise was needed, the scientific councilsthe AHA were contacted for writing committee represen-

tives. Representation was provided or facilitated by theouncils on Cardiovascular Nursing, Cardiovascular Surgeryd Anesthesia, Cardiovascular Radiology and Intervention,d Clinical Cardiology, Council for High Blood Pressure

esearch, and Stroke Council.

.3. Document Review and Approvalhis document was reviewed by 3 outside reviewers nomi-ted by the ACCF and 2 outside reviewers nominated by theHA, as well as 1 or 2 reviewers from each of the followingganizations: the AATS, ACP, ACEP, ACR, ASA, SCA,
AI, SIR, STS, and the SVM. It was also reviewed by 6

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dividual content reviewers—2 content reviewers from theCCF Catherization Committee and 1 content reviewer frome ACCF Interventional Council. All reviewer RWI infor-ation was collected and distributed to the writing committeed is published in this document (see Appendix 2).This document was approved for publication by the gov-ning bodies of the ACCF and the AHA and the AATS,CR, ASA, SCA, SCAI, SIR, STS, and SVM and wasdorsed by the North American Society for Cardiovascularaging.

.4. Scope of the Guidelinehe term “thoracic aortic disease” encompasses a broad rangedegenerative, structural, acquired, genetic-based, and trau-

atic disease states and presentations. According to the

ble 1. Applying Classification of Recommendations and Level

*Data available from clinical trials or registries about the usefulness/efficacyyocardial infarction, history of heart failure, and prior aspirin use. A recommendany important clinical questions addressed in the guidelines do not lend them

a very clear clinical consensus that a particular test or therapy is useful or†In 2003, the ACCF/AHA Task Force on Practice Guidelines developed a

commendations have been written in full sentences that express a completee rest of the document (including headings above sets of recommendations),rease readers’ comprehension of the guidelines and will allow queries at the

enters for Disease Control and Prevention death certificate ag

ta, diseases of the aorta and its branches account for 43 00047 000 deaths annually in the United States (2). The precisember of deaths attributable to thoracic aortic diseases isclear. However, autopsy studies suggest that the presenta-n of thoracic aortic disease is often death due to aortic

ssection (AoD) and rupture, and these deaths account forice as many deaths as attributed to ruptured abdominalrtic aneurysms (AAAs) (3). The diagnosis of acute thoracicoD or rupture is often difficult and delayed, and errors inagnosis may account for deaths otherwise attributed tordiac arrhythmia, myocardial infarction (MI), pulmonarybolism, or mesenteric ischemia.The University HealthSystem Consortium (UHC) is an

liance of more than 100 academic medical centers andfiliate hospitals. UHC’s Clinical DataBase/Resource Man-

ence

rent subpopulations, such as sex, age, history of diabetes, history of priorth Level of Evidence B or C does not imply that the recommendation is weak.o clinical trials. Even though randomized trials are not available, there may.suggested phrases to use when writing recommendations. All guideline, such that a recommendation, even if separated and presented apart fromstill convey the full intent of the recommendation. It is hoped that this willual recommendation level.

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mes for performance improvement. The UHC provided theriting committee with a summary of recent informationsed on ICD-9 codes for thoracic aortic disease–relatedspitalizations from the Clinical DataBase/Resource Man-er (Tables 2A and 2B). This data table demonstrates a highmber of hospital discharges (more than 135 000) fororacic, abdominal, thoracoabdominal, and “unspecified”rtic aneurysms in the 5-year period between 2002 and07. Subcategories include those with dissection, those withpture, and those with neither. In the most recent 1-yearriod assessed (fourth quarter 2006 through third quarter07), there were nearly 9000 cases representing all patients

ith thoracic aortic disease discharged from UHC hospitals.lthough these data are unavailable for the entire United

ble 2A. ICD-9 Procedure Codes For Aortic Aneurysms

rtic Aneurysmtegory Dissection Ruptured

No Mention ofRupture

oracic 441.01 441.1 441.2

dominal 441.02 441.3 441.4

oracoabdominal 441.03 441.6 441.7

rtic (unspecified) 441.00 441.5 441.9

Table courtesy of UHC Clinical DataBase/Resource Manager.

ble 2B. Number of Discharges by Year by Category of Aortic AC Clinical Database*

rtic Aneurysmtegory 2002q4–2003q3 2003q4–2004q3 2004q4–2005q3

oracic

Dissection 1607 1683 2028

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3086 4026 4953

Subtotal 4880 5934 7200

dominal



No mention ofture

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Subtotal 13 279 14 567 17 349

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Subtotal 1824 1899 2256

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tal No. ofses

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UHC indicates University HealthSystem Consortium.*Note: Year-to-year increases are due in part to changes in number of report

Table courtesy of UHC Clinical DataBase/Resource Manager.

ates, they provide important estimates of the magnitude ofe prevalence of thoracic aortic disease. Additional informa-n regarding the patient admission source, particularly those

ith acute presentations, is pertinent to the discussion regard-g interinstitutional transfer (see Section 17.2).Most patients with significant thoracic aortic disease willdirected to specialized practitioners and institutions. How-

er, the importance of early recognition and prompt treat-ent and/or referral for various thoracic aortic diseases by allalthcare professionals provides the rationale for this docu-ent. This guideline will attempt to provide the practitionerith a sufficient description of background information,agnostic modalities, and treatment strategies so that appro-iate care of these patients can be facilitated and betterderstood. The goal of this guideline is to improve the healthtcomes and quality of life for all patients with thoracicrtic disease.Other practice guidelines developed by ACCF and AHAdress the management of patients with cardiac and vascularseases. The ACCF/AHA guidelines on peripheral arterialsease (4) include recommendations for lower extremity,nal, mesenteric, and abdominal aortic diseases. Data stan-rds on this topic are currently in development, as is aactice guideline on extracranial carotid and vertebral artery

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seases. The ACCF/AHA guidelines are published in theurnal of the American College of Cardiology and Circula-n and are available on both the ACC (www.acc.org) and

HA (my.americanheart.org) Web sites.This guideline includes diseases involving any or all partsthe thoracic aorta with the exception of aortic valve

seases (5) and includes the abdominal aorta when contigu-s thoracic aortic diseases are present. Specific disease statese described in the following sections, and the reader isferred to the glossary of terminology in Section 1.5 andppendix 3 for abbreviations used throughout the guideline.The reader will note that several topics or referenced areasay appear more than once throughout the guideline. Thisill appear to be redundant to those who choose to read thetire document, but the writing committee believed thatcause of the multidisciplinary nature of and organizationalvolvement in this project, individuals representing specificsciplines may choose to read and extract portions of thecument for their own use. Inclusion of the narrative textd references was thought to be appropriate to facilitate aore complete understanding for these disciplines and indi-duals. Accordingly, the organization of the guideline iseant to be less of a textbook presentation of the variouspics but rather a more clinically oriented document appli-ble to a variety of disciplines.

4.1. Critical Issuess the writing committee developed this guideline, severalitical issues emerged:

Thoracic aortic diseases are usually asymptomatic and noteasily detectable until an acute and often catastrophiccomplication occurs. Imaging of the thoracic aorta withcomputed tomographic imaging (CT), magnetic resonanceimaging (MR), or in some cases, echocardiographic exam-ination is the only method to detect thoracic aortic diseasesand determine risk for future complications (see Section 4).Radiologic imaging technologies have improved in termsof accuracy of detection of thoracic aortic disease. How-ever, as the use of these technologies has increased, so alsohas the potential risk associated with repeated radiationexposure, as well as contrast medium–related toxicity.Whether these technologies should be used repeatedly as awidespread screening tool is discussed in Section 4. Inaddition, the writing committee formulated recommenda-tions on a standard reporting format for thoracic aorticfindings as discussed in Section 4.5.Imaging for asymptomatic patients at high risk based onhistory or associated diseases is expensive and not alwayscovered by payers.For many thoracic aortic diseases, results of treatment forstable, often asymptomatic, but high-risk conditions are farbetter than the results of treatment required for acute andoften catastrophic disease presentations. Thus, the identi-fication and treatment of patients at risk for acute andcatastrophic disease presentations (e.g., thoracic AoD,thoracic aneurysm rupture) prior to such an occurrence areparamount to eliminating the high morbidity and mortality
associated with acute presentations (see Section 8.1).
A subset of patients with acute AoD are subject to missedor delayed detection of this catastrophic disease state.Many present with atypical symptoms and findings, mak-ing diagnosis even more difficult (see Sections 8.1.4 and8.6). This issue has come under greater medical-legalscrutiny, and specific cases have been widely discussed inthe public domain. Widespread awareness of the variedand complex nature of thoracic aortic disease presentationshas been lacking, especially for acute AoD. Risk factorsand clinical presentation clues are noted in Section 8.1.4.The collaboration and cosponsorship of multiple medicalspecialties in the writing of this guideline will provideunique opportunities for widespread dissemination ofknowledge to raise the level of awareness among allmedical specialties.There is rapidly accumulating evidence that genetic alter-ations or mutations predispose some individuals to aorticdiseases (see Section 5). Therefore, identification of thegenetic alterations leading to these aortic diseases has thepotential for early identification of individuals at risk. Inaddition, biochemical abnormalities involved in the pro-gression of aortic disease are being identified throughstudies of patients’ aortic samples and animal models ofthe disease (6,7). The biochemical alterations identified inthe aortic tissue have the potential to serve as biomarkersfor aortic disease. Understanding the molecular pathogen-esis may lead to targeted therapy to prevent aortic disease.Medical and gene-based treatments are beginning to showpromise for reducing or delaying catastrophic complica-tions of thoracic aortic diseases (see Section 9.2).As noted in Section 18, there are several areas wheregreater resources for research and both short- and long-term outcomes registries are needed.

.5. Glossary of Terms and Abbreviationssed Throughout Guideline

neurysm (or true aneurysm): a permanent localized dila-tation of an artery, having at least a 50% increase indiameter compared with the expected normal diameter ofthe artery in question. Although all 3 layers (intima,media, and adventitia) may be present, the intima andmedia in large aneurysms may be so attenuated that insome sections of the wall they are undetectable.

seudoaneurysm (or false aneurysm): contains blood re-sulting from disruption of the arterial wall with extrava-sation of blood contained by periarterial connective tissueand not by the arterial wall layers (see Section 8.4). Suchan extravascular hematoma that freely communicates withthe intravascular space is also known as a pulsatinghematoma (8–10).

ctasia: arterial dilatation less than 150% of normal arterialdiameter.

rteriomegaly: diffuse arterial dilatation involving severalarterial segments with an increase in diameter greaterthan 50% by comparison to the expected normal arterial
diameter.
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horacoabdominal aneurysm (TAA): aneurysm involvingthe thoracic and abdominal aorta (see Section 9.2.2.3).

bdominal aortic aneurysm (AAA): aneurysm involvingthe infradiaphragmatic abdominal aorta.

ortic dissection (AoD): disruption of the media layer of theaorta with bleeding within and along the wall of the aorta.Dissection may, and often does, occur without an aneu-rysm being present. An aneurysm may, and often does,occur without dissection. The term “dissecting aorticaneurysm” is often used incorrectly and should be re-served only for those cases where a dissection occurs in ananeurysmal aorta (see Section 8.1).

. The Thoracic Aorta

.1. The Normal Aortahe thoracic aorta is divided into 4 parts: the aortic roothich includes the aortic valve annulus, the aortic valvesps, and the sinuses of Valsalva); the ascending aortahich includes the tubular portion of the ascending aortaginning at the sinotubular junction and extending to theachiocephalic artery origin); the aortic arch (which beginsthe origin of the brachiocephalic artery and is the origin ofe head and neck arteries, coursing in front of the trachea and

the left of the esophagus and the trachea); and thescending aorta (which begins at the isthmus between theigin of the left subclavian artery and the ligamentumteriosum and courses anterior to the vertebral column, anden through the diaphragm into the abdomen).The normal human adult aortic wall is composed of 3

yers, listed from the blood flow surface outward (Figure 1):

tima: endothelial layer on a basement membrane withminimal ground substance and connective tissue.

edia: bounded by an internal elastic lamina, a fenestratedsheet of elastic fibers; layers of elastic fibers arrangedconcentrically with interposed smooth muscle cells;bounded by an external elastic lamina, another fenestrated
sheet of elastic fibers. ap
dventitia: resilient layer of collagen containing the vasavasorum and nerves. Some of the vasa vasorum canpenetrate into the outer third of the media.

.2. Normal Thoracic Aortic Diameter1991, the Society for Vascular Surgery created a table

able 3) describing the normal diameter of the adult thoracicrta based on CT and chest x-ray (12).Since then, it has been recognized that the “normal aorticameter” is influenced by a number of factors, includingtient age, sex, and body size; location of aortic measure-ent; method of measurement; and the robustness and type ofaging methods used. Hannuksela et al (13) noted that

ameter increased by 0.12 to 0.29 mm/y at each leveleasured by CT for 41 men and 36 women aged 18 to 82ars (Figure 2). Aortic diameter for men was larger than thatr women, but the difference decreased with age. Body massdex also affected aortic diameter by 0.27 mm (0.14 to 0.44m) per unit of body mass index (13).Aortic diameter gradually tapers downstream from the

nuses of Valsalva. Hager et al (14) examined 46 men and 24omen without cardiovascular disease (age range 1 to 89

ble 3. Normal Adult Thoracic Aortic Diameters

horacic AortaRange of Reported

Mean (cm)Reported SD

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2.40 to 2.44 0.32 CT

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CT indicates computed tomographic imaging; CXR, chest x-ray; and NA, not

Figure 1. Aortic pathology associated with thoracicaortic aneurysm involving the ascending aorta. Allpanels are identically oriented with the adventitia atthe top and the intima at the bottom. H&E staining ofaortic sections from a control (a) and a patient (b) witha TAA demonstrates medial degeneration with thefragmentation of elastic fibers, accumulation of pro-teoglycans, and regions of smooth muscle cell loss.Movat staining of aortic sections from control (c) andpatient with an aneurysm (d) shows fragmentation ofelastic fibers (stained black), loss of smooth musclecells (cells stained red and nuclei stained violet), andaccumulation of proteoglycans (stained blue) in themedial layer. 40� magnification; scale bars represent500 mcg. H&E indicates hematoxylin and eosin; andTAA, thoracic aortic aneurysm. Modified fromMilewicz et al (11).

plicable. Reprinted with permission from Johnston et al (12).

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ars; mean age 50.2 years) using helical CT (Figure 3). Forese patients, there was no correlation with weight, height, ordy surface area, but aortic diameter increased with age and

as larger for men than for women (14).Two-dimensional echocardiography has been used to de-e the “normal” range for aortic diameter at the sinuses of

alsalva in different age categories (and stratified by bodyrface area) (15). Adjusting for 2 of the key determinants ofrtic diameter allows a more precise characterization ofrtic size in otherwise healthy individuals (15) (Figure 4).gain, age and sex affected aortic root diameter, but thefluence of sex was neutralized when diameter was indexedbody surface area (Table 4).These tables and definitions help define the presence orsence of a thoracic aortic aneurysm and help define thereshold for considering further treatment for such patients.owever, patients with certain genetic syndromes and abnor-al tissue morphology may in fact have a normal aorticameter at the time of acute AoD rupture (see Section 5.1.2).nother challenge relates to abnormal morphology of onertic segment compared with another. For example, if theameter of the ascending aorta exceeds the diameter of the

gure 2. Normal diameter and upper limit of ascending andscending aorta related to age. Reprinted with permission from

annuksela et al (13).

rta at the level of the sinuses Valsalva, even if both areithin normal range, then the ascending aorta is considered to

enlarged. To adjust for body habitus variation, the use ofrtic diameter indexed to height has been reported to betterdicate surgical timing than might be recommended fromrtic diameter alone for an otherwise asymptomatic patientith Marfan syndrome or bicuspid aortic valve (16). When-er possible, the writing committee has inserted aorticameter thresholds for further action, whether the action isr continued surveillance or for endovascular or surgicaltervention.

. Thoracic Aortic Histopathology

.1. Atherosclerosistherosclerosis is characterized by intimal lesions calledheromata, or atheromatous or fibrofatty plaques, whichotrude into the arterial lumen and weaken the underlyingedia often associated with calcification. With aging, pres-ce of risk factors, and genetic predisposition, thisogresses to complicated lesions with surface defects, hem-rhage, and/or thrombosis. A 1995 consensus documentom the AHA defines the types and histologic classes ofherosclerosis (17) (Figure 5).Thoracic aortic atherosclerosis is less common than ab-minal aortic atherosclerosis, but the clinical importance iseat. Clinical presentations and problems associated withrtic atherosclerosis and atheroma are discussed extensivelySection 11.

.2. Aneurysms and Dissectionshe pathology associated with thoracic aortic aneurysms andssections was initially termed cystic medial necrosis butis term is a misnomer; the disease is not associated withcrosis of the aorta or with cyst formation. Aortic aneurysmstopathology, more accurately termed medial degeneration,characterized by disruption and loss of elastic fibers and

creased deposition of proteoglycans (Figure 1). Typically,ere are areas of loss of smooth muscle cells in the aorticedia, but whether there is a total loss of smooth muscle cellsthe aortic wall is not clear. There can be atherosclerosis

sions present, but again, these changes are typically super-

Figure 3. Mean aortic diameters (in cm) at variouslevels measured by helical CT in 70 adults. Thin linesrepresent �2 SDs, representing 95% reference area.CT indicates computed tomographic imaging; and SD,standard deviation. Reprinted with permission fromHager et al (14).

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posed on medial degenerative disease. Although medialgeneration was initially described as a noninflammatorysease, recent literature supports the presence of inflamma-ry cell infiltration in this disease (18,19).The biochemical pathways and proteins involved withedial degeneration have not been clearly delineated.owever, multiple studies have found increased immuno-aining for a subset of matrix metalloproteinases (MMPs)

the media of thoracic aortic aneurysms, particularlyMP-2 and MMP-9 (20 –23). Immunostaining of aorticedia from patients with Marfan syndrome has demon-rated increases in MMP-2 and MMP-9, which weresociated with smooth muscle cells at the borders of areasmedial degeneration and on the surface of disrupted

astic fibers. Elevated MMP-2 and MMP-9 immunostain-g has been demonstrated in ascending aneurysms fromtients with either tricuspid or bicuspid aortic valves1,23) and inconsistently in ascending aortic tissue fromtients with tricuspid aortic valves (22). These 2 MMPse known to have elastolytic activity. Variable expression

MMPs and tissue inhibitors of MMPs has also beenmonstrated in aortic tissue of patients with Marfanndrome versus patients without Marfan syndrome (24).lthough accumulation of proteoglycans in the aorticedia is another consistent finding in thoracic aortic

ble 4. Sex Differences in Aortic Root Dimensions in Adults

rtic RootAbsolute Values

(cm) Men P Value

nulus 2.6�0.3 �0.001

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Adapted from Roman et al (15).
eurysms, no studies have determined why this accumu-tion occurs or whether these are causative in nature.Medial degeneration is also associated with focal loss ofscular smooth muscle cells. Although there are regionssmooth muscle cell loss, morphometric analysis of

rtic tissue has suggested that hyperplastic cellular re-odeling of the media in ascending thoracic aortic aneu-sms may be an initial adaptive response to minimizecreased wall stress resulting from vascular dilatation5). More recent studies of the aortic pathology associatedith myosin heavy chain 11, smooth muscle (MYH11), andtin, alpha 2, smooth muscle aorta (ACTA2) mutationsading to ascending aortic aneurysms demonstrate aperplastic response by smooth muscle cells in the aorticedia. The aortic media in aneurysm tissue taken fromtients harboring mutations in these genes demonstratedcal hyperplasia associated with smooth muscle cells thatere remarkable for a lack of structured orientationrallel to the lumen of the aorta, but instead, the smoothuscle cells were oriented randomly with respect to oneother (26,27).

.3. Vasculitis and Inflammatory Diseasesvariety of inflammatory vasculitides may also result in

oracic aortic disease. These include giant cell arteritis

Figure 4. Sinus of Valsalva diameter, bybody surface area. Left, The 95% normalconfidence limits for aortic root diameter atthe sinuses of Valsalva in relation to bodysurface area in adults �40 years of age.Right, The 95% normal confidence limits forthe proximal ascending aortic diameter in re-lation to body surface area in adults �40years of age. SEE indicates standard error ofthe estimate. Reprinted with permission fromRoman et al (15).

menIndexed

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CA), Takayasu arteritis, and Behçet disease (see Section. The pathophysiology of GCA shares important featuresith Takayasu arteritis (28). T-cell clonal expansion sug-sts a specific antigenic response, which currently re-ains unelucidated. The inflammatory response, whichgins in the adventitial layer, is marked by augmentedtokine and MMP production causing granuloma forma-

on. Granuloma formation both shields the vessel from theciting antigen and causes vessel destruction (29). Behçetsease is a vasculitis affecting both arteries and veins, ofl sizes.

. Imaging Modalities

.1. Recommendations for Aortic Imagingchniques to Determine the Presence and

rogression of Thoracic Aortic DiseaseASS I

Measurements of aortic diameter should be taken at reproduc-ible anatomic landmarks, perpendicular to the axis of bloodflow, and reported in a clear and consistent format (see Table5). (Level of Evidence: C)For measurements taken by computed tomographic imagingor magnetic resonance imaging, the external diametershould be measured perpendicular to the axis of blood flow.For aortic root measurements, the widest diameter, typi-cally at the mid-sinus level, should be used. (Level ofEvidence: C)For measurements taken by echocardiography, the internal diam-eter should be measured perpendicular to the axis of blood flow.For aortic root measurements, the widest diameter, typically at
the mid-sinus level, should be used. (Level of Evidence: C) ulc
Abnormalities of aortic morphology should be recognized andreported separately even when aortic diameters are withinnormal limits. (Level of Evidence: C)The finding of aortic dissection, aneurysm, traumatic injuryand/or aortic rupture should be immediately communicated tothe referring physician. (Level of Evidence: C)Techniques to minimize episodic and cumulative radiationexposure should be utilized whenever possible. (30,31) (Levelof Evidence: B)

Figure 5. Atherosclerotic lesions. Flow dia-gram in center column indicates pathways inevolution and progression of human athero-sclerotic lesions. Roman numerals indicatehistologically characteristic types of lesionsdefined at the left of the flow diagram. Thedirection of the arrows indicates the se-quence in which characteristic morphologiesmay change. From Type I to Type IV,changes in lesion morphology occur primarilybecause of increasing accumulation of lipid.The loop between Types V and VI illustrateshow lesions increase in thickness whenthrombotic deposits form on their surfaces.Thrombotic deposits may form repeatedlyover varied time spans in the same locationand may be the principal mechanism forgradual occlusion of medium-sized arteries.Adapted from Stary et al (17).

ble 5. Essential Elements of Aortic Imaging Reports

The location at which the aorta is abnormal (see Section 2).

The maximum diameter of any dilatation, measured from the externalwall of the aorta, perpendicular to the axis of flow, and the length of theaorta that is abnormal.

For patients with presumed or documented genetic syndromes at risk foraortic root disease measurements of aortic valve, sinuses of Valsalva,sinotubular junction, and ascending aorta.

The presence of internal filling defects consistent with thrombus oratheroma.

The presence of IMH, PAU, and calcification.

Extension of aortic abnormality into branch vessels, including dissectionand aneurysm, and secondary evidence of end-organ injury (e.g., renalor bowel hypoperfusion).

Evidence of aortic rupture, including periaortic and mediastinalhematoma, pericardial and pleural fluid, and contrast extravasation fromthe aortic lumen.

When a prior examination is available, direct image to image comparisonto determine if there has been any increase in diameter.

IMH indicates intramural hematoma; and PAU, penetrating atherosclerotic
er.

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If clinical information is available, it can be useful to relateaortic diameter to the patient’s age and body size. (Level ofEvidence: C)

efinitive identification or exclusion of thoracic aortic dis-se or one of its anatomic variants requires dedicated aorticaging. Selection of the most appropriate imaging study

ay depend on patient-related factors (i.e., hemodynamicability, renal function, contrast allergy) and institutionalpabilities (i.e., rapid availability of individual imagingodalities, state of the technology, and imaging specialistpertise). Consideration should be given to patients withrderline abnormal renal function (serum creatinine greateran 1.8 to 2.0 mg/dL)—specifically, the tradeoffs betweene use of iodinated intravenous contrast for CT and thessibility of contrast-induced nephropathy, and gadoliniuments used with MR and the risk of nephrogenic systemicrosis (32).Radiation exposure should be minimized (31,33–36). The

sk of radiation-induced malignancy is the greatest in neo-tes, children, and young adults (31). Generally, above thee of 30 to 35 years, the probability of radiation-inducedalignancy decreases substantially (30,31). For patients whoquire repeated imaging to follow an aortic abnormality, MRay be preferred to CT. MR may require sedation due tonger examination times and tendency for claustrophobia.CT as opposed to echocardiography can best identifyoracic aortic disease, as well as other disease processes thatn mimic aortic disease, including pulmonary embolism,ricardial disease, and hiatal hernia. After intervention oren surgery, CT is preferred to detect asymptomatic post-ocedural leaks or pseudoaneurysms because of the presencemetallic closure devices and clips.We recommend that external aortic diameter be reportedr CT or MR derived size measurements. This is importantcause lumen size may not accurately reflect the externalrtic diameter in the setting of intraluminal clot, aortic wallflammation, or AoD. A recent refinement in the CT mea-rement of aortic size examines the vessel size using anterline of flow, which reduces the error of tangentialeasurement and allows true short-axis measurement ofrtic diameter. In contrast to tomographic methods,hocardiography-derived sizes are reported as internal diam-er size. In the ascending aorta, where mural thrombus ineurysms is unusual, the discrepancy between the internald external aortic diameters is less than it is in the descend-g or abdominal aorta, where mural thrombus is common.andardization of aortic diameter measurements is importantthe planning of endovascular treatment of an aneurysm,

here the diameter of the aorta in the seal zone must beatched to the diameter of an endograft. Here, the choice ofternal or external diameter is specified by the manufacturerthe endograft.

.2. Chest X-Rayoutine chest x-ray may occasionally detect abnormalities ofrtic contour or size that require definitive aortic imaging.

hest x-ray often serves as a part of the evaluation of patients th

ith potential acute AoD, primarily to identify other causespatient’s symptoms, but also as a screening test to identifydings due to a dilated aorta or bleeding. However, chestray is inadequately sensitive to definitively exclude theesence of AoD in all except the lowest-risk patients anderefore rarely excludes the disease. Pooled data from 10udies places the predictive sensitivity of a widened medi-tinum or an abnormal aortic contour associated with sig-ficant thoracic aortic disease at 64% and 71%, respectively7). In the same analysis, however, including all abnormaldiographic findings increased the sensitivity to 90%, sug-sting that a completely normal chest x-ray does lower theelihood of AoD and may provide meaningful clinical

formation in very low-risk patients (37). The presence of aidened mediastinum or other radiographic findings sugges-e of thoracic aortic disease increases the likelihood of

oD, particularly among patients who lack a clear alternativeurce for their symptoms. In a blinded prospective study of6 patients who underwent evaluation for acute thoracicrtic disease, the specificity of chest x-ray for aortic pathol-y was 86% (38).For patients with chest trauma, chest x-ray is a poorreening test for the diagnosis of aortic injury (39–41). Aarply demarcated normal mediastinal contour is sometimesed to exclude mediastinal hematoma (suggesting a wideediastinum, abnormal mediastinal contour, left apical cap,ss of the aortic knob, depression of the left main bronchus,d deviation of an indwelling esophageal tube). However,ese signs of hemomediastinum are more often false positivean true positive for aortic injury (40). Figure 6 illustrates thermal appearance of the thoracic aorta, and its components,a posteroanterior chest x-ray.

.3. Computed Tomographic ImagingT scanning has been used for more than 2 decades toentify acute AoD and to diagnose and measure other

gure 6. Chest x-ray of a normal thoracic aorta. Arrows indi-te arch, and arrowheads show ascending and descendingrta.

oracic aortic diseases (42) (see Section 5.1). Advantages

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clude near universal availability—the ability to image thetire aorta, including lumen, wall, and periaortic regions; toentify anatomic variants and branch vessel involvement;d to distinguish among types of acute aortic syndromes

.e., intramural hematoma [IMH], penetrating atheroscleroticcer [PAU], and acute AoD)—and the short time required tomplete the imaging process and the 3-dimensional data.

lectrocardiogram (ECG)-gated techniques have made itssible to generate motion-free images of the aortic root andronary arteries, similar to coronary CT angiographic imag-g.Reports of newer-generation multidetector helical CTanners show sensitivities of up to 100% and specificities of% to 99% (43–46). Data from the International Registry of

cute Aortic Dissection (IRAD) show that for patients withrtic dissection, CT was the initially used diagnostic modal-

in 61% of patients and transthoracic echocardiographyTE) and/or transesophageal echocardiography (TEE) wased first in 33% of patients. Whether this was because the

gure 7. Axial CT images demonstrating the value of noncon-st images. Top, Precontrast image demonstrates a high-at-

nuation aortic hematoma indicating an acute aortic event. Bot-m, Images obtained with intravenous contrast materialmonstrate the contrast-filled aortic lumen and the hematomaa relatively lower attenuation band. CT indicates computed

mographic imaging.

st test was insufficient to diagnose dissection or because pr

ditional information about cardiac and aortic valve functionas required is unclear (47).The sequence for CT performed in the potential setting ofute AoD generally would include a noncontrast study totect subtle changes of IMH (Figure 7), followed by antrast study to delineate the presence and extent of thessection flap, identify regions of potential malperfusion,d demonstrate contrast leak indicating rupture. Imaging ofe vascular tree from the thoracic inlet to the pelvis,cluding the iliac and femoral arteries, provides sufficientformation to plan surgical or endovascular treatment, ifeded. Prompt interpretation and communication of findingsthe appropriate treating physicians are essential in the acutetting.For trauma patients, the sensitivity, specificity, and accu-cy of contrast-enhanced multidetector CT for traumaticrtic injury are 96%, 99%, and 99%, respectively (48–50).

he negative predictive value of contrast-enhanced CT ap-oaches 100% in many studies, most with in-hospitalllow-up of negative CT examinations (36,48,51,52)igure 8).An outcome study of 278 patients undergoing contrast-hanced CT for blunt chest trauma revealed 6 patients withrta/great vessel injury and confirmed the 100% negative

gure 8. Traumatic aortic rupture secondary to a motor vehicleash. Top, Axial CT image demonstrates a traumatic injury witheudoaneurysm (T) in the proximal descending thoracic aorta,merous bilateral rib fractures, and small bilateral pleural effu-

ons, with no significant mediastinal hematoma. Bottom, Stillage through the thorax further demonstrates the extent of thertic trauma. See the accompanying full cine video for the bot-m panel. CT indicates computed tomographic imaging.

edictive value in the remaining patients using an extensive

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view of medical databases with a median follow-up of 20.5onths (53).Regarding other thoracic aortic diseases, CT has beenown to have a 92% accuracy for diagnosing abnormalitiesthe thoracic aorta, in a series of examinations that includedthoracic aneurysms, 3 ruptured thoracic aortic aneurysms,

PAUs, 5 AoDs, and 2 pseudoaneurysms. In addition, CTrrectly predicted the need for hypothermic circulatoryrest during surgical repair in 94% of patients (54). Forngenital and inflammatory conditions of the thoracic aorta,e literature is primarily descriptive, and accuracy data aret available.

3.1. Computed Tomographic Imaging Techniquelthough nonhelical CT scanners are capable of diagnosingoracic aortic disease, the technique used is axial step-and-oot technology. Inherent limitations include slow scaneed, relatively poor spatial resolution given thicker colli-ation used to extend the needed craniocaudal coverage, andherently a noncontiguous dataset. Any patient motion, eveninimal, between the acquisition of each image or cluster ofages creates a stair-step artifact on multiplanar anddimensional renderings. Helical CT scanners of 4 andeater detector rows consistently provide volumetric acqui-tions of the thoracic aorta. Scanners with 16 and greatertector rows provide isotropic resolution in the x, y, and zes, which allows the datasets to be reconstructed in thetimum imaging plane best suited to any individual vessel.Technical parameters recommended for image reconstruc-n are slices of 3-mm or less thickness with a reconstruction

terval of 50% or less than the slice thickness at 50% oreater overlap, tube rotation of 1 second or less, and 120 to
0 kVp (55). ECG gating is particularly useful for ascending ro
rtic disease, eliminating motion artifact at the aortic rootat can simulate an AoD (Figure 9), and allowing evaluationaortic valve morphology and function, as well as evalua-n of the proximal coronary arteries. If appropriately ac-ired, an aorta CT and a complete CT coronary angiogramn be obtained in 1 CT acquisition. For AoD, the scanverage should start above the aortic arch and extend at leastthe aortoiliac bifurcation, and probably the groin. This isportant to determine both branch vessel involvement, suchlesion extension into the abdominal aorta and iliac arteriesd characterization and diagnosis of malperfusion syn-ome, and to evaluate access for percutaneous repair whenansluminal therapy is being considered. (For further infor-ation on technique parameters and anatomic coverage, seee online-only Data Supplement.)The CT angiographic acquisition uses intravenous contrastlivered at rate of 3 to 5 mL/s by a power injector andually followed by a saline bolus. The total volume ofntrast used should be kept as low as possible, to no greateran 150 mL.Although axial sections remain the mainstay of interpreta-n, 2- and 3-dimensional reconstructions, such as maximum

tensity projection, multiplanar and curved multiplanar ref-mations, and volume rendering, may augment interpreta-n and improve communication of the findings (54). To ourowledge, it has not been scientifically shown that the use ofese tools increases diagnostic accuracy or diagnostic con-ence among specialists. For example, in 1 study multipla-r reconstructions when added to axial images aloneanged the interpretation in only 1 case (54). However,dimensional reconstruction is likely to play an important

Figure 9. Mimic of aortic dissection created bymotion of the aortic root. Top left, Image at thelevel of the right pulmonary artery demonstrates anormal descending thoracic aorta and pseudodis-section of the ascending aorta due to motion arti-fact that occurs on non–ECG-gated CT examina-tions (arrow). Top right, Image at the aortic rootshows a double contour to the aortic root that maysimulate a dissection flap (arrow). Bottom, Still im-age through the thoracic aorta further delineatesthe extent of the motion artifact. See the accom-panying full cine video. CT indicates computedtomographic imaging; and ECG, electrocardio-gram.

le in the planning of surgical or endovascular approaches.

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.4. Magnetic Resonance ImagingR has been shown to be very accurate in the diagnosis oforacic aortic disease, with sensitivities and specificities thate equivalent to or may exceed those for CT and TEE4,58–62). Like CT, MR provides a multiplanar evaluationthe thoracic aorta, and the examination can be extended inverage to include the length of the aorta and branch vessels,

om skull base to the toes. Advantages of MR include theility to identify anatomic variants of AoD (IMH and PAU),sess branch artery involvement, and diagnose aortic valvethology and left ventricular dysfunction without exposinge patient to either radiation or iodinated contrast. Disadvan-ges include prolonged duration of imaging acquisitionring which the patient is inaccessible to care providers;ability to use gadolinium contrast in patients with renalsufficiency; contraindication in patients with claustropho-a, metallic implants, or pacemakers; and lack of widespreadailability on an emergency basis. Although time-resolvedR techniques are improving, MR often cannot clearlyaracterize the relationship of an intimal flap and aortic root

ructures, specifically the coronary arteries. Likely as a resultthese considerations, data from IRAD found that MR was the

ast-used imaging study, used in only 1% of patients as theitial diagnostic study (63). For traumatic injury and congenitald inflammatory conditions of the thoracic aorta, the literatureprimarily descriptive, and accuracy data are not available.

4.1. Magnetic Resonance Imaging Techniquecomprehensive MR examination of the thoracic aorta mayclude many components, including black blood imaging,ay include basic spin-echo sequences, noncontrast whiteood imaging, contrast-enhanced MR angiography usingdolinium based agents, and phase-contrast imaging. MR ofe thoracic aorta in the acute setting can be shortened to meetgent patient assessment needs. The use of each is describedore specifically later. MR examinations have become faster,ith advances in gradient hardware that have significantlyduced repetition times, resulting in ultrafast MR angiogra-y techniques. However, MR examinations remain 2 to 4es longer than CT examinations. MR terminology across

aging vendors is less consistent than that of CT; therefore,aging sequences are described later as general types ofquences without vendor-specific details (58,59).

4.2. Black Blood Imaginglack blood imaging, using spin-echo sequences, is used toaluate aortic anatomy and morphology (such as aortic sized shape), and the aortic wall for hematoma or other causesthickening such as vasculitis. They may be repeated after

e administration of gadolinium-based contrast agents toentify wall enhancement. These sequences should use ECGting at end-diastole and may be performed with or withoutuble inversion recovery techniques that null the signal fromood. These sequences generate 2-dimensional images thatn be obtained in the axial, sagittal, and coronal planes, asell as the oblique sagittal or “candy cane” view. T1-eighted gradient echo sequences may be used in place ofack blood images and are usually performed both befored after contrast administration. They can be obtained in the
ial, sagittal, and coronal planes. ar
4.3. Noncontrast White Blood Imaginghis is performed using either basic gradient echo sequences

the more advanced balanced steady-state free precessionchniques (SSFP) that are T2*weighted and generate imagesith subsecond temporal resolution. Signal is generated fromood, making it appear white in the absence of contrast.FP techniques use ultrashort repetition times that requireR scanners with high-performance gradients. For the latter,in-plane resolution of approximately 2.0�1.5 mm2 can betained using a repetition time of 3.2 ms, TE of 1.6 ms, flipgle of 60 to 70 degrees, and a 256�256 matrix. SSFPchniques can be performed to generate 2-dimensional,dimensional, and cine images; the 2-dimensional imagese usually performed in the axial, sagittal, and coronal planesan interleaved manner and use ECG gating with triggeringend-diastole to generate images in less than 500 ms that dot require breath holding. Cine SSFP sequences require 7- tosecond breath holding and are usually only performed atecific anatomic locations of interested, as determined by thedings on the previously generated images. For patients whonnot hold their breath, cine SSFP sequences can be per-rmed without using ECG gating or breath holding but withbstantially lower spatial and temporal resolution.

4.4. Contrast-Enhanced Magneticesonance Angiographyr the thoracic aorta, contrast-enhanced MR angiography isually performed with ECG gating. Although this increasesquisition time, it provides motion-free images of the aorticot and ascending aorta. For patients without a contraindi-tion to receiving a gadolinium-based contrast agent,ntrast-enhanced MR angiography is often the sequence ofoice from which most of the diagnostic information istained. Contrast-enhanced MR angiography images aretained as a 3-dimensional volumetric dataset, which can beanipulated and viewed in much the same manner as CTan datasets. Advances in MR, particularly gradient strength,ve markedly reduced the acquisition times of contrast-hanced MR angiography possible to subsecond acquisitiones. This temporally resolved subsecond contrast-enhanced

R angiography is particularly useful in critically ill patientspatients who cannot hold their breath, where motion

tifact can degrade other sequences that take longer to acquire,ith the tradeoff being a reduction of in-plane resolution.

4.5. Phase Contrast Imaginghese sequences are usually performed to evaluate gradientsross an area of stenosis, across an intimal or cardiac valve.age contrast is produced by differences in velocity. Images

e obtained using ECG gating or triggering. Two-mensional images are generated that center on the area ofncern. Peak flow and velocity measurements may be calcu-ted, and time–flow and time–velocity curves are generated.

.5. Standards for Reporting of the Thoracicorta on Computed Tomography andagnetic Resonance Imagingiewing and measuring are best accomplished at a picture
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gure 10. Left brachiocephalic vein mimics an intramural he-atoma on CT. Axial CT image demonstrates a low-attenuationescent of material anterior to the innominate artery. CT indi-tes computed tomographic imaging.

The writing committee believes that specific qualitative andantitative elements are important to include in reports (Table 5).Diameter measurements taken from axial images are inher-tly incorrect unless the artery being measured is perfectlyigned in cross section on the image (Figure 11). It is preferablemake diameter measurements perpendicular to the longitudi-l or flow axis of the aorta to correct for the variable geometrythe aorta. Suggested standard anatomic locations are noted in

gure 12. The use of standardized measurements helps mini-ize errant reports of significant aneurysm growth due tochnique or interreader variability in measuring technique.

.6. Angiographyngiography provides accurate information about the site ofssection, branch artery involvement, and communication ofe true and false lumens (60,61). Additionally, angiographicd catheter-based techniques allow for evaluation and treat-ent of coronary artery (62) and aortic branch (visceral and

b artery) disease, as well as assessment of aortic valve andft ventricular function (60,63).Disadvantages of angiography compared with other lessvasive modalities include 1) not being universally availablecause it requires the presence of an experienced physicianperform the study; 2) being an invasive procedure that ise consuming and requires exposure to iodinated contrast;having poor ability to diagnose IMH given a lack of

Figure 11. Markedly tortuous aorta with thoraco-abdominal aortic aneurysm demonstrated on (A)3-dimensional shaded surface display rendering. Band C represent incorrect measurement of theaorta on standard coronal and axial images re-spectively, while D is an image of the aorta per-pendicular to the centerline or axis of the aorta,with the arrow demonstrating the correct locationfor diameter measurement, which in this case was7.8 cm.

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minal disruption; 4) potentially producing false negativesults when a thrombosed false lumen prevents adequateacification to identify the dissection; and 5) reportednsitivities and specificities of angiography for the evalua-n of acute AoD that are slightly lower than those for the

her less invasive modalities (42,64,65). Thus, CT, MR, andEE have replaced catheter-based angiography as the first-e diagnostic tests to establish the presence of the acutertic syndrome (60,63,66,67). Multidetector CT has alsorgely replaced angiography for the anatomic studies re-ired for treatment planning and monitoring of aortic dis-se (68).

.7. Echocardiographyhe aorta and its major branches can be visualized withhocardiography using a variety of imaging fields as well asethods of ultrasound. The suprasternal view is best forsualizing the aortic arch, whereas the aortic root andcending aorta are best seen in the left (and sometimes right)rasternal projection. When involvement of the innominatetery, left subclavian artery, or left common carotid artery isspected, orthogonal and longitudinal scanning planes arelpful. Imaging of the descending thoracic aorta is lesssily accomplished with echocardiography compared withher imaging modalities. Abdominal scan planes can be used

gure 12. Normal anatomy of the thoracoabdominal aorta withandard anatomic landmarks for reporting aortic diameter asstrated on a volume-rendered CT image of the thoracic aorta.

T indicates computed tomographic imaging. Anatomic loca-ns: 1, Aortic sinuses of Valsalva; 2, Sinotubular junction; 3,id ascending aorta (midpoint in length between Nos. 2 and 4);Proximal aortic arch (aorta at the origin of the innominate ar-

ry); 5, Mid aortic arch (between left common carotid and sub-avian arteries); 6, Proximal descending thoracic aorta (beginsthe isthmus, approximately 2 cm distal to left subclavian ar-

ry); 7, Mid descending aorta (midpoint in length between Nos.and 8); 8, Aorta at diaphragm (2 cm above the celiac axis ori-n); 9, Abdominal aorta at the celiac axis origin. CT indicatesmputed tomographic imaging.

visualize the caudal descending aorta. lu

In general, TEE is superior to TTE for assessment of theoracic aorta. Through the use of multiplane image acquisi-n, 3-dimensional Doppler TEE is safe and can be per-rmed at the bedside, with a low risk of complications (lessan 1% overall, less than 0.03% for esophageal perforation),ost of which are related to conscious sedation (69,70).econstruction of the aorta can be performed. Intraoperativee of TEE is noted in Sections 14.2.3 and 14.2.4.

7.1. Echocardiographic Criteria for Thoracicortic Aneurysmshe echocardiographic diagnosis of thoracic aortic aneu-sms is determined on demonstration of aortic enlargementlative to the expected aortic diameter, based on age- anddy size–adjusted nomograms (Figures 2, 3, 4, and 13). Forher aortic segments, such nomograms have not been devel-ed, so aortic dilatation is diagnosed when the aorta exceedsgenerally agreed to standard diameter (Table 3) or when aven aortic segment is larger than contiguous aortic seg-ent(s) of apparently normal size. Beyond establishing theesence of aortic enlargement, echocardiography may revealsociated cardiac pathology that suggests the underlyingiology of the aortic disease (e.g., bicuspid aortic valve).

7.2. Echocardiographic Criteria forortic Dissectionhe echocardiographic diagnosis of an AoD requires the iden-cation of a dissection flap separating true and false lumensigure 14). However, one of the major limitations of both TTEd TEE is the frequent appearance of artifacts that mimic assection flap (Figure 15). These usually arise from a mirrorage or reverberation artifact that appears as a mobile linearhodensity overlying the aortic lumen. It is therefore essentialat the echocardiographer make certain to distinguish truessection flaps from such artifacts. The first step is to confirme presence of the dissection flap from several angles and fromveral transducer locations. The second step is to confirm thate dissection flap has a motion independent of surroundingructures and that the apparent flap is contained within the aortic

gure 13. Transthoracic echocardiogram of a patient witharfan syndrome with mitral valve prolapse and 4-cm ascend-g aortic aneurysm.

men (i.e., does not pass through the aortic wall in any view).

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he third step is to usecolor-flow Doppler to demonstratefferential flow on the 2 sides of the dissection flap. Often onen visualize 1 or more sites of intimal tears, with color-flowoppler demonstrating sites of flow between the 2 lumens. Theue lumen typically exhibits expansion during systole andllapse during diastole. Additional signs of the true lumenclude little or nospontaneous echocardiographic contrast, sys-lic jets directed away from the lumen, and forward flow duringstole. The false lumen often exhibits diastolic expansion,idence of spontaneous contrast, complete or partial thrombo-

s, and reversed, delayed, or absent blood flow. Imaging criteriar distinguishing the true and false lumens have also beenscribed for intravascular ultrasound (71) and CT (72). Featuresat characterize the false lumen of an acute dissection includee wedgelike angle (the “beak sign”) where the dissection flapeets the aortic wall, the presence of strandlike structurescobwebs”) in the lumen, and the lack of a laminar structure ine outer wall of the lumen.Associated echocardiographic findings of clinical importance

clude identification of a pericardial effusion. Indeed, it is oftenssible to see a layer of echogenic material within the pericar-al fluid that is indicative of hemopericardium. Echocardiogra-y can provide important information about right and left

gure 14. Arch aneurysm with dissection flap. Top, Arch dis-ction, 2-dimensional view. Bottom, Arch dissection (arrow)

ith color-flow Doppler margination.

ntricular function and myocardial ischemia based on assess- w

ent of left ventricular segmental wall motion. On TEE, one canentify the ostia of the 2 coronary arteries and detect involve-ent by the dissection flap. Echocardiography also identifies theesence of aortic regurgitation and permits grading of itsverity. On TEE, the mechanisms of acute aortic regurgitationn be defined, and these data can be used to guide the surgeon’sforts to spare the valve at the time of aortic repair.

7.2.1. DIAGNOSTIC ACCURACY OF ECHOCARDIOGRAPHY

R AORTIC DISSECTION

TE has a sensitivity of 77% to 80% and a specificity of 93%96% for identification of proximal AoD. For distal AoD,

e sensitivity of TTE is lower. TEE improves the diagnosticcuracy substantially, particularly when a patient’s bodyape, chest wall, or concomitant pulmonary disease restrictse transthoracic windows for aortic imaging. With TEE,nsitivity for proximal AoD is 88% to 98% with a specificity90% to 95% (46). A 2006 meta-analysis that evaluated the

agnostic utility of TEE in suspected thoracic AoD included0 patients from 10 different studies. TEE was shown toth accurately identify and rule out acute AoD with sensi-ities and specificities of 98% (95% CI 95% to 99%) and% (95% CI 92% to 97%), respectively (46).

Major advantages of TEE include its portability (allowingr bedside patient evaluation), rapid imaging time, and lackintravenous contrast or ionizing radiation. Additionally,

ssection-related cardiac complications can be evaluatedcluding aortic regurgitation, proximal coronary artery in-lvement, and the presence of tamponade physiology. Dis-vantages of TEE include a potential lack of availabilityarticularly at small centers and during off hours) anddation requirements that may include endotracheal intuba-n (73). The accuracy of TEE can be quite operatorpendent. For the very distal ascending aorta and theoximal aortic arch, TEE may be limited by a blind spotused by interposition of the trachea and left main bronchustween the esophagus and aorta. Small, circumscribed AoDsIMHs in this region are less well visualized by TEE.

nother limitation of TEE is its inability to visualize thedominal aorta; if there is concern of a malperfusionndrome, an abdominal CT may also be required.

7.2.2. DIAGNOSTIC ACCURACY OF ECHOCARDIOGRAPHY

R ACUTE INTRAMURAL HEMATOMA

H of the aorta has a distinctly different appearance onhocardiography. In contrast to classic AoD, in IMH there is noobile intimal flap within the aortic lumen, and in most casese aortic lumen has a relatively normal round appearance.stead, in IMH there is thickening of the aortic wall that ispically crescentic in shape and extends along a length of therta. In some cases it can be difficult to distinguish the wallickening of IMH from diffuse aortic atherosclerosis or muralrombus lining an aortic aneurysm. However, atheroma andural thrombus protrude from the aortic intima into the lumen,us giving both the aortic wall and the lumen an irregular shape,hereas in IMH the inner lining of the aortic lumen remainsooth. Moreover, in the presence of intimal calcification, intramu-

l thrombus will present as thickening external to calcification,
hereas mural thrombus will be internal to calcification.

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7.2.3. ROLE OF ECHOCARDIOGRAPHY IN FOLLOWING PA-

ENTS WITH CHRONIC AORTIC DISEASE

iven that TEE is a semi-invasive procedure; it is usually noteferred for surveillance of patients with thoracic aortic dis-ses. Also, although TTE is noninvasive, its failure to visualizensistently and measure accurately the tubular portion of thecending thoracic aorta is problematic. It is not typically used tollow aneurysms in that aortic segment. However, because TTEes accurately visualize the aortic root, its primary role as anaging method for serial follow-up is in patients with aortic

sease limited to the root, particularly those with Marfanndrome. It is also used, often in conjunction with CT or MR,observe patients with concomitant structural heart disease,

ch as bicuspid aortic valve, mitral valve prolapse, cardiomeg-

y, or cardiomyopathy.

. Genetic Syndromes Associated Withhoracic Aortic Aneurysms and Dissection

.1. Recommendations for Genetic SyndromesASS I

An echocardiogram is recommended at the time of

gure 15. Artifact mimicking dissection. Top left, 2-dimensional vct not seen in this view.

diagnosis of Marfan syndrome to determine the aortic

root and ascending aortic diameters and 6 months thereaf-ter to determine the rate of enlargement of the aorta. (Levelof Evidence: C)Annual imaging is recommended for patients with Marfansyndrome if stability of the aortic diameter is documented. Ifthe maximal aortic diameter is 4.5 cm or greater, or if theaortic diameter shows significant growth from baseline,more frequent imaging should be considered. (Level ofEvidence: C)Patients with Loeys-Dietz syndrome or a confirmed geneticmutation known to predispose to aortic aneurysms and aorticdissections (TGFBR1, TGFBR2, FBN1, ACTA2, or MYH11)should undergo complete aortic imaging at initial diagnosisand 6 months thereafter to establish if enlargement is occur-ring. (74–77) (Level of Evidence: C)Patients with Loeys-Dietz syndrome should have yearly mag-netic resonance imaging from the cerebrovascular circulationto the pelvis. (23,78,79) (Level of Evidence: B)Patients with Turner syndrome should undergo imaging of theheart and aorta for evidence of bicuspid aortic valve, coarcta-tion of the aorta, or dilatation of the ascending thoracic aorta.(80) If initial imaging is normal and there are no risk factors foraortic dissection, repeat imaging should be performed every 5to 10 years or if otherwise clinically indicated. If abnormalitiesexist, annual imaging or follow-up imaging should be done.

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ASS IIa

It is reasonable to consider surgical repair of the aorta in alladult patients with Loeys-Dietz syndrome or a confirmed TG-FBR1 or TGFBR2 mutation and an aortic diameter of 4.2 cm orgreater by transesophageal echocardiogram (internal diame-ter) or 4.4 to 4.6 cm or greater by computed tomographicimaging and/or magnetic resonance imaging (external diame-ter). (78) (Level of Evidence: C)For women with Marfan syndrome contemplating pregnancy, itis reasonable to prophylactically replace the aortic root andascending aorta if the diameter exceeds 4.0 cm. (74) (Level ofEvidence: C)If the maximal cross-sectional area in square centimeters ofthe ascending aorta or root divided by the patient’s height inmeters exceeds a ratio of 10, surgical repair is reasonablebecause shorter patients have dissection at a smaller size and15% of patients with Marfan syndrome have dissection at asize less than 5.0 cm. (16,76,81) (Level of Evidence: C)

ASS IIb

In patients with Turner syndrome with additional risk factors,including bicuspid aortic valve, coarctation of the aorta,and/or hypertension, and in patients who attempt to becomepregnant or who become pregnant, it may be reasonable toperform imaging of the heart and aorta to help determine therisk of aortic dissection. (Level of Evidence: C)

1.1. Marfan Syndromearfan syndrome is a heritable disorder of the connectivesue with a high penetrance but variable expression. Ap-oximately 25% of patients do not have a family history andpresent new cases due to sporadic mutations for thendition. Marfan syndrome results from mutations in the

BN1 gene, with over 600 mutations currently entered intoe FBN1 mutation database causing Marfan syndrome orlated conditions (http://www.umd.be/). The FBN1 genecodes fibrillin-1, a large glycoprotein that is secreted fromlls and deposited in the extracellular matrix in structureslled microfibrils. Microfibrils are found at the periphery ofastic fibers, including the elastic fibers in the medial layer ofe ascending aorta, and in tissues not associated with elasticers (82). Only 12% of FBN1 mutations causing Marfanndrome have been observed more than once in unrelateddividuals, a fact that complicates using mutational detectionr diagnosis. A second locus for Marfan syndrome, termedFS2, was recently identified to be caused by mutations ine transforming growth factor-beta type II receptorGFBR2) (83). The phenotype of this locus may overlap for

oeys-Dietz syndrome. The criteria for Marfan syndrome issed primarily on clinical findings in the various organstems affected in the Marfan syndrome, along with familystory and FBN1 mutations status (84).The cardinal features of Marfan syndrome involve therdiovascular, ocular, and skeletal systems. Patients witharfan syndrome are highly predisposed to thoracic aorticeurysm and/or dissection, with virtually every patient withe syndrome having evidence of aortic disease at some pointring their lifetime. Other cardiovascular manifestations
clude valvular disease, primarily mitral valve prolapse and w
gurgitation (85). Aortic regurgitation can result from dis-rtion of the aortic valve cusps by an enlarged aortic root.he skeletal manifestations reflect overgrowth of the longnes and include arachnodactyly, dolichostenomelia, kypho-oliosis, dolichocephaly, and pectus deformities. Abnormal-es in the connective tissues are also manifested as jointxity, recurrent or incisional hernias, striae atrophica, andral ectasia (84). The ocular manifestation that is bothnsitive and fairly specific for Marfan syndrome is ectopiantis or lens dislocation. The presence of ectopia lentis is articularly useful clinical finding to differentiate Marfanndrome from Loeys-Dietz syndrome (78).Most patients with Marfan syndrome present with dilata-n of the aortic root/ascending aorta or Type A dissection.ternal aortic diameter measured at the sinuses of Valsalvaovides a baseline for future evaluations because this is thertic segment that dilates in Marfan syndrome. Of note,hocardiographic studies measure the internal diameter,hereas most patients undergo definitive imaging by CTd/or MR, which measures the external diameter (expectedbe 0.2 to 0.4 cm larger than internal diameter), and externalameter is the measurement used in most cases to determinee threshold for surgical repair. There is growing awareness

the importance of relating this measurement to normallues based on age and body surface area (15) (Table 4). Theverity of the aortic disease is related to the degree andgment length of aortic dilatation with dilatation limited toe sinuses of Valsalva having a less malignant prognosisan dilatation that extends to the aortic arch (86). Afteragnosis, follow-up imaging studies are recommended at 6onths and then annually if stability is documented based one high likelihood of aortic disease progression. In mostses, TTE can be used to monitor the size of the sinuses ofalsalva.A subset of patients present with Type B dissection, and a

re patient will present with AAA. The poor outcome oftients with Marfan syndrome with acute Type B dissectionss led some to advocate early surgical repair (87).Studies addressing the efficacy of beta blockade in patients

ith Marfan syndrome have shown slower aortic root growth,wer cardiovascular end points (defined as aortic regurgita-n, dissection, surgery, heart failure, or death), and im-oved survival (88). Patients continue to enlarge their aortad dissect on therapy, so such medication does not precludee need for routine imaging and prophylactic aortic repairhen the diameter of the aorta warrants repair. It is alsoportant to note that significant aortic root dilatation isrrelated negatively with therapeutic response (88). Recent

udies in a mouse model of Marfan syndrome with aorticsease similar to that seen in humans showed that treatmentith losartan normalized aortic root growth (6), and a clinicalial using losartan in Marfan syndrome patients under the age

25 years is in progress (89,90) (see Section 9.2.1.1).Surgical repair of the dilated aortic root/ascending aorta fortients with Marfan syndrome is usually performed at areshold of a external diameter of 5.0 cm (91), smaller thanat for other patients because of the greater tendency foroD at a smaller diameter (see Section 9.2.2.1). Factors that
ill prompt repair at an external diameter of less than 5.0 cm
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e rapid growth defined as greater than 0.5 cm/y, familystory of AoD at a diameter less than 5.0 cm, or the presencesignificant aortic regurgitation.After prophylactic repair of the ascending aorta, the archd descending aorta are sites for later-onset aneurysms andssections in patients with Marfan syndrome, prompting theed for routine imaging of the arch and descending aorta.rvival in patients with Marfan syndrome has been signifi-ntly improved with medical and surgical management ofe aortic disease (76,92,93). The David valve sparing reim-antation operation for suitable patients undergoing electivertic root surgery at centers with a high volume of theseses has become standard practice (76,92–99), althoughme have reported less-optimal long-term results with valve-aring procedures (100,101).Pregnant patients with Marfan syndrome are at increased

sk for AoD if the aortic diameter exceeds 4 cm (102) (seection 10). All women with Marfan syndrome warrant

equent cardiovascular monitoring throughout pregnancyd into the puerperium. Limited data on the treatment ofomen with Marfan syndrome who experience dissectionsring pregnancy suggest a better outcome with cesareanction with concomitant aortic repair (103).

1.2. Loeys-Dietz Syndromehe Loeys-Dietz syndrome is an autosomal dominant aorticeurysm syndrome with involvement of many other systems8,104). Loeys-Dietz syndrome results from mutations inther the transforming growth factor receptor Type I or IIGFBR1 or TGFBR2) genes and the diagnosis is confirmedrough mutational analysis of these genes (105).The disease is characterized by the triad of arterial tortu-sity and aneurysms, hypertelorism and bifid uvula or cleftlate, or a uvula with a wide base or prominent ridge on it.

he arterial tortuousity is most commonly observed in thead and neck vessels but can occur in other vessels. Thesetients also have a spectrum of other features, which includee following: velvety and translucent skin, craniosynostosis,alar hypoplasia, retrognathia, blue sclera, patent ductusteriosus, skeletal features similar to Marfan syndrome,ral ectasia, atrial septal defects, developmental delay,rvical spine abnormalities, and joint laxity. The vascularsease in these patients is particularly aggressive with aean age of death of 26 years (78). Most patients have aorticot aneurysms (98%) that lead to AoD. Because there areultiple reports of AoD occurring in patients with Loeys-ietz when the aortic diameter was less than 5.0 cm, repair iscommended at smaller diameters (78) (see Section 5.1). Forung children with severe systemic manifestations of Loeys-

ietz syndrome, specifically prominent craniofacial featuresat are associated with more severe aortic disease, once thertic diameter exceeds the 99th percentile for age and thertic valve annulus reaches 1.8 to 2.0 cm, prophylacticrgery allows for the placement of a graft of sufficient size tocommodate growth. Of note, echocardiographic examina-ns that measure the internal aortic diameter were used in

ese studies to determine the threshold for surgical repair.Patients with Loeys-Dietz also develop aneurysms of other
ssels (53%), leading to the recommendation that they have de
arly MR imaging from the cerebrovascular circulation toe pelvis. Current studies indicate that aggressive surgicalanagement of the aneurysms in these patients can behieved with few complications (23,78). Surgical proce-res in patients with Loeys-Dietz syndrome are not compli-ted by tissue fragility (23,79).

1.3. Ehlers-Danlos Syndrome, Vascular FormType IV

he vascular form of Ehlers-Danlos syndrome is a raretosomal dominant disorder characterized by easy bruising,in skin with visible veins, characteristic facial features, andpture of arteries, uterus, or intestines. Rupture of thestrointestinal tract is more likely to occur prior to arterialpture, and the majority of patients survive the gastrointes-al rupture (106). Most of the fatal complications are causedarterial rupture, with most deaths attributable to arterial

ssections or ruptures involving primarily the thoracic ordominal arteries, including AoDs and ruptures. Theseterial ruptures lead to reduced life expectancy, with theedian survival of only 48 years, and often no aneurysms arecumented. If the rupture of an artery is life threatening, itn be surgically repaired, but tissue fragility, tendency tomorrhage extensively, and poor wound healing may com-icate the surgical repair (107). Whether there is a role fore repair of unruptured aneurysms in patients with thisndrome is not clear, which is in contrast to Loeys-Dietzndrome, where a role for prophylactic surgical repair ofeurysms is already well established. Nevertheless, whenese patients present with AoD or aortic root aneurysms,ccessful aortic surgery can be achieved with careful han-ing of tissues and resewing of anastomoses with pledgetedtures (76). Noninvasive vascular imaging is preferred astal complications have been associated with invasive imag-g in these patients (106). The outcome of pregnancy inomen with Ehlers-Danlos syndrome is poor because ofpture of the gravid uterus and vessel rupture at delivery orthe postpartum period. The diagnosis of vascular Ehlers-

anlos syndrome is based on DNA or protein studies iden-ying a defect in type III collagen, encoded by the COL3A1ne.

1.4. Turner Syndromeurner syndrome is defined as complete or partial absence ofsex chromosome in a phenotypic female, most commonly, X. Short stature and ovarian failure are the most prevalentding, but women with Turner syndrome have an increasedrdiovascular mortality rate from both structural and isch-ic heart disease, especially AoD (108,109). Between 10%

d 25% of patients with Turner syndrome have a bicuspidrtic valve. Aortic coarctation is present in approximately

of patients. Determining aortic dilatation in patients withurner syndrome is difficult because aortic dilatation is based

body surface area so the aortas of patients with Turnerndrome are expected to be smaller than those of the generalpulation because of the patient’s short stature. If one
fines aortic dilatation as an ascending-to-descending aortic

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ameter ratio of greater than 1.5, then 33% of women withurner syndrome had aortic dilatation (110).The average age of AoD in Turner syndrome was 31 years,d less than half of the patients survived the event (111).ata indicate a population-based AoD incidence of 36:1000 Turner syndrome years (1.4% among individuals with

urner syndrome) compared with 6:100 000 in the generalanish population (112). Therefore, the risk of AoD is muchwer in patients with Turner syndrome compared withtients with Marfan syndrome or Loeys-Dietz syndrome.

he majority of dissections in women with Turner syndromecur in patients with known risk factors for dissection, suchcardiovascular malformations (bicuspid aortic valve or

arctation of the aorta), systemic hypertension, or both.herefore, the evidence base regarding the value of screeningr aortic disease in women with Turner syndrome is notailable. However, there appears to be an increased risk forssection in these women, suggesting that imaging of theart, aorta, and pulmonary veins at the time of diagnosisight be valuable (80,113). For patients with no risk factorsr AoD (bicuspid aortic valve, coarctation, dilated aorta),-evaluation of the aorta has been suggested every 5 to 10ars or if clinically indicated (e.g., attempting pregnancy14) or transition to an adult clinic). Patients with riskctors for AoD should undergo more frequent imaging.ecently, studies have not shown an effect of growth hor-one treatment in women with Turner syndrome on eithercending or descending aortic diameter (113). In addition,udies have not found any evidence of left ventricularpertrophy in patients with Turner syndrome who were

eated with growth hormone (115,116).

1.5. Other Genetic Syndromes With Increased Riskr Thoracic Aortic Aneurysms and Dissectionssubstantial proportion of patients with Ehlers-Danlos syn-ome who do not have the vascular form also have aorticot dilatation, but the progression of this dilatation to AoD isre (76,117). Similarly, patients with congenital contracturalachnodactyly or Beals syndrome due to mutations in FBN2ve had aortic root enlargement without documented pro-ession to dissection (118,119).There are other genetic syndromes that have multiple

ports or documentation of thoracic aortic aneurysms lead-g to Type A dissections. There are multiple case reports ofoD in patients with autosomal dominant polycystic kidneysease (120,121). Although AoD is a complication of auto-mal dominant polycystic kidney disease, it is less commonan cerebral aneurysms leading to subarachnoid hemorrhagethis population. There is insufficient information to gauge

e value of routine or screening imaging for these patients.Similar to autosomal dominant polycystic kidney disease,

ere are multiple reports in the literature of patients withoonan syndrome who are experiencing AoDs (122–124).he value of imaging or routine monitoring of these patientsunknown. A review of 200 patients with Alagille syndromeso identified thoracic aortic disease in a small subset of
ese patients (125). TG
1.6. Recommendations for Familial Thoracic Aorticneurysms and DissectionsASS I

Aortic imaging is recommended for first-degree relatives ofpatients with thoracic aortic aneurysm and/or dissection toidentify those with asymptomatic disease. (126,127) (Level ofEvidence: B)If the mutant gene (FBN1, TGFBR1, TGFBR2, COL3A1, ACTA2,MYH11) associated with aortic aneurysm and/or dissection isidentified in a patient, first-degree relatives should undergocounseling and testing. Then, only the relatives with thegenetic mutation should undergo aortic imaging. (Level ofEvidence: C)

ASS IIa

If one or more first-degree relatives of a patient with knownthoracic aortic aneurysm and/or dissection are found to havethoracic aortic dilatation, aneurysm, or dissection, then imag-ing of second-degree relatives is reasonable. (126) (Level ofEvidence: B)Sequencing of the ACTA2 gene is reasonable in patients witha family history of thoracic aortic aneurysms and/or dissec-tions to determine if ACTA2 mutations are responsible for theinherited predisposition. (26,27,77,78,128,129) (Level of Ev-idence: B)

ASS IIb

Sequencing of other genes known to cause familial thoracicaortic aneurysms and/or dissection (TGFBR1, TGFBR2,MYH11) may be considered in patients with a family historyand clinical features associated with mutations in thesegenes. (26,27,77,78,128,129) (Level of Evidence: B)If one or more first-degree relatives of a patient with knownthoracic aortic aneurysm and/or dissection are found to havethoracic aortic dilatation, aneurysm, or dissection, then refer-ral to a geneticist may be considered. (Level of Evidence: C)

genetic basis of nonsyndromic familial thoracic aorticeurysms and dissection has only recently been defined.milial aggregation studies of patients referred for repair oforacic aortic aneurysm and dissection that did not have anetic defect have indicated that between 11% and 19% ofese patients have a first-degree relative with thoracic aorticeurysms and dissection (127,130). Patients with a familystory of thoracic aortic aneurysm and dissection present atyounger mean age than do sporadic patients but at a

gnificantly older age than patients with Marfan or Loeys-ietz syndrome. The disease is primarily inherited in antosomal dominant manner with decreased penetrance pri-arily in women and variable expression (131). Theseapping studies have firmly established that there is signif-ant genetic heterogeneity for familial thoracic aortic aneu-sm and dissection (i.e., many different genes can be mutatedd cause the same clinical condition) (27,77,129,132–134).

he causative gene has been identified for the following loci:AD2 is due to TGFBR2 mutations, the mutant gene at 16p

the MYH11 gene, and the TAAD4 defective gene is ACTA2.The defective gene at the TAAD2 locus for familial

oracic aortic aneurysms and dissection was identified as
FBR2, which is the same gene that is mutated in approx-

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ately two thirds of patients with Loeys-Dietz syndrome.enetic testing of unrelated families with familial thoracicrtic aneurysm and dissections demonstrated that TGFBR2utations were only present in 1% to 5% of families (26). Allfamilies had mutations that affected TGFBR2 arginine 460

the receptor, suggesting that missense mutation wassociated with familial thoracic aortic aneurysm and dissec-n (i.e., genotype-phenotype correlation). Although the

ajority of vascular disease in these families involved thecending aorta, affected family members also had descend-g aortic disease and aneurysms of other arteries, includingrebral, carotid, and popliteal aneurysms. It is notable thatmilar to Loeys-Dietz syndrome, AoDs occur in patientsith TGFBR2 mutation at diameters less than 5.0 cm, leadingthe recommendation that aortic repair be considered at a

ternal diameter by echocardiography of 4.2 cm or greater8) (see Section 5.1).A large French family with thoracic aortic aneurysm and

ssection associated with patent ductus arteriosus was usedmap the defective gene causing this phenotype to 16p

35). The defective gene at this locus was identified as theooth muscle cell–specific myosin heavy chain 11YH11), a major protein in the contractile unit in smooth

uscle cells (77). Subsequent analysis of DNA from 93related families with thoracic aortic aneurysm and dissec-n failed to identify any MYH11 mutations. Sequencing

NA from 3 unrelated families with thoracic aortic aneurysmd dissection associated with patent ductus arteriosus iden-ed MYH11 mutations in 2 of these families (26); the

maining family had a TGFBR2 mutation as the cause of theoracic aortic aneurysm and dissection and patent ductusteriosus. Therefore, MYH11 mutations are responsible formilial thoracic aortic aneurysm and dissection associatedith patent ductus arteriosus and a rare cause of familialoracic aortic aneurysm and dissection.A defective gene at the locus 10q23–24 was identified in a

rge family with multiple members with thoracic aorticeurysm and dissection as ACTA2, which encodes the theooth muscle-specific alpha-actin, a component of thentractile complex and the most abundant protein in vascu-r smooth muscle cells (27,136). Approximately 15% of

ble 6. Gene Defects Associated With Familial Thoracic Aortic

fective Gene Leading toamilial Thoracic Aorticeurysms and Dissection

Contribution to Familial ThoracicAortic Aneurysms and

Dissection

FBR2 mutations 4%

YH11 mutations 1%

TA2 mutations 14%

ACTA2 indicates actin, alpha 2, smooth muscle aorta; MYH11, smooth muscceptor type II.

milies with thoracic aortic aneurysm and dissection have un

CTA2 mutations (27). Features identified in some familiesith ACTA2 mutation included livedo reticularis and iriscculi, although the prevalence of these features has noten determined. The majority of affected individuals pre-nted with acute Type A or B dissections or Type Bssections, with 16 of 24 deaths occurring due to Type Assections. Two of 13 individuals experienced Type Assections with a documented ascending aortic diameter lessan 5.0 cm. AoDs occurred in 3 individuals under 20 yearsage, and 2 women died of dissections postpartum. Finally,

young men had Type B dissection complicated by ruptureaneurysm formation at the ages of 13, 16, and 21 years.Identification of the underlying genetic mutation leading to

milial thoracic aortic aneurysms and dissections providesitical clinical information for the family. First, only familyembers who harbor mutations need to be routinely imagedr aortic disease. Second, identification of the underlyingutation may lead to different management of the aorticsease, as is the case for TGFBR2 mutations. In addition tooviding information to families, identification of genesading to familial thoracic aortic aneurysms and dissectionss emphasized the roles of smooth muscle contractilenction in preventing aortic diseases (11). Individuals whodergo genetic testing for thoracic aortic disease shouldceive genetic counseling prior to the testing to explain theplications for the testing for their medical follow-up andplications for family members.

.2. Summaryhe genes leading to nonsyndromic forms of aortic aneu-sms and dissections are in the early stages of identification.ables 6 and 7 summarize the current clinical featuressociated with mutations in these genes and recommenda-ns for when to sequence these genes in families with

ultiple members with familial thoracic aortic aneurysm andssection.Given the familial risk of thoracic aortic aneurysms,reening the proband’s first-degree relatives with appropri-e imaging studies is indicated in the absence of identifica-n of the defective gene leading to the disease.Because thoracic aortic disease is typically asymptomatic

sm and Dissection

Associated Clinical Features Comments on Aortic Disease

Thin, translucent skin Multiple aortic dissectionsdocumented at aorticdiameters �5.0 cm

Arterial or aortic tortuosity

Aneurysm of arteries

Patent ductus arteriosus Patient with documenteddissection at 4.5 cm

Livedo reticularis Two of 13 patients withdocumented dissections

�5.0 cmIris flocculi

Patent ductus arteriosus

Bicuspid aortic valve

fic beta-myosin heavy chain; and TGFBR2, transforming growth factor-beta

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her family members can potentially prevent prematureaths. Most syndromes and familial forms of thoracic aorticsease are inherited in an autosomal dominant manner.herefore, an individual with an inherited predisposition tooracic aortic aneurysm and dissections has up to a 50% riskpassing on this predisposition to their children, which is thesis for genetic evaluation of the offspring. In addition,

blings and parents of the patient need to be evaluated forssible predisposition to thoracic aortic aneurysm and dis-ctions. Because of the variable age of onset of aortic diseasefamilial thoracic aortic aneurysms and dissections, the

riting committee believes that imaging of family membersrisk of the disease every 2 years is warranted.

. Other Cardiovascular Conditionsssociated With Thoracic Aorticneurysm and Dissection

.1. Recommendations for Bicuspid Aorticalve and Associated Congenital VariantsAdults

ASS I

First-degree relatives of patients with a bicuspid aortic valve,premature onset of thoracic aortic disease with minimal riskfactors, and/or a familial form of thoracic aortic aneurysm anddissection should be evaluated for the presence of a bicuspidaortic valve and asymptomatic thoracic aortic disease. (Level

ble 7. Genetic Syndromes Associated With Thoracic Aortic An

netic Syndrome Common Clinical Features Gen

rfan syndrome Skeletal features (see text) FBN1Ectopia lentisDural ectasia

eys-Dietz syndrome Bifid uvula or cleft palate TGFBRmArterial tortuosity

Hypertelorism

Skeletal features similar to MFS

Craniosynostosis

Aneurysms and dissections of otherarteries

lers-Danlos syndrome,scular form

Thin, translucent skin COL3

Gastrointestinal rupture

Rupture of the gravid uterus

Rupture of medium-sized to largearteries

rner syndrome Short stature 45,X

Primary amenorrhea

Bicuspid aortic valve

Aortic coarctation

Webbed neck, low-set ears, low hairline,broad chest

AoD indicates aortic dissection; COL3A1, type III collagen; CT, computed tomoaging; TEE, transesophageal echocardiogram; TGFBR1, transforming growth fa

*The defective gene at a second locus for MFS is TGFBR2 but the clinical

of Evidence: C) dr

All patients with a bicuspid aortic valve should have both theaortic root and ascending thoracic aorta evaluated for evi-dence of aortic dilatation. (137–140) (Level of Evidence: B)

icuspid aortic valves is the most common congenital abnor-ality affecting the aortic valve and the aorta and is found in

to 2% of the population (137). Nine percent of patientsve family members who also have bicuspid aortic valves41). The ACC/AHA Valvular Heart Disease Guidelinesecifically address this condition (5). Of importance to thisideline, bicuspid aortic valves can be inherited in familiesan autosomal dominant condition and may be associated

ith thoracic aortic aneurysm formation. It is important tote that in these families, members can have thoracic aorticeurysms in the absence of bicuspid aortic valves (142). Thelves are prone to either aortic valve regurgitation, mostmmonly seen in younger patients, or aortic valve stenosis,ore common in older patients. Bicuspid aortic valve repairr regurgitation has excellent long-term results, an importantnsideration in the absence of prosthetic aortic valve alter-tives in this young population (99,139,140,143). The mostmmon site of fusion of the leaflets is at the left and rightaflet commissure, and less so at the right noncoronaryaflet commissure. The latter is typically more often associ-ed with aortic valve stenosis. In a study of 2000 patients ate Cleveland Clinic who underwent bicuspid aortic valvergery, 20% had concurrent ascending aortic aneurysms thatquired repair (139,140). AoD is also common, and as many

15% of patients with acute AoD have bicuspid aorticlves (144), a frequency more common than Marfan syn-

and Dissection

ct Diagnostic Test Comments on Aortic Disease

ns* Ghent diagnostic criteria Surgical repair when the aorta reaches5.0 cm unless there is a family history

of AoD at �5.0 cm, a rapidlyexpanding aneurysm or presence orsignificant aortic valve regurgitation

DNA for sequencing

BR1 DNA for sequencing Surgical repair recommended at anaortic diameter of �4.2 cm by TEE

(internal diameter) or 4.4 to �4.6 cmby CT and/or MR (external diameter)

ions DNA for sequencing Surgical repair is complicated byfriable tissues

Noninvasive imaging recommendedDermal fibroblasts for

analysis of type IIIcollagen

pe Blood (cells) forkaryotype analysis

AoD risk is increased in patients withbicuspid aortic valve, aortic coarctation,

hypertension, orpregnancy

imaging; FBN1, fibrillin 1; MFS, Marfan syndrome; MR, magnetic resonanceta receptor type I; TGFBR2, transforming growth factor beta receptor type II.ype as MFS is debated.

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lves with an aortic diameter less than 5 cm, reminiscent oftients with Marfan syndrome in whom 15% had AoD at a

ze less than 5 cm (16,143).The writing committee discussed the potential need toreen relatives of patients who have undergone aortic valveplacement before age 70, as these younger patients wereore likely to have had bicuspid aortic valve as their primarythology. However, 40% of women and around one third ofen over age 70 undergoing aortic valve replacement havecuspid aortic valve disease (145). The yield from screeninglatives of younger patients with bicuspid aortic valvesease compared with relatives of older patients is notown.

.2. Aberrant Right Subclavian Arteryberrant right subclavian artery, which arises as the fourthanch from the aorta, courses behind the esophagus inproximately 80% of patients and causes dysphagia in manytients (146–149). The dysphagia usually occurs in adults ase artery enlarges (Kommerell diverticulum) (150,151). Inost adult patients, the aorta is also abnormal and is prone toeurysm formation, dissection, and rupture. Surgical treat-ent in adults involves resection of the aneurysmal segmentthe subclavian artery (the diverticulum) and the adjacent

rta and replacement of the aorta with a graft (152). Anternative treatment is exclusion of the right subclaviantery origin and adjacent aorta using an aortic endograft,though long-term follow-up of this endovascular approachnot available and the compression and aneurysm growth

ay continue (153). The distal normal segment of thebclavian artery is usually not reimplanted into the descend-g aorta directly but rather revascularized using an interpo-tion graft or carotid-subclavian bypass. Alternatively, thebclavian artery can be ligated or coiled distal to theeurysm, implanting the distal segment into the adjacentrotid artery in the neck and thus restoring flow to the uppertremity and the ipsilateral vertebral artery (148,149,152).

.3. Coarctation of the Aortaoarctation of the aorta is a relatively common abnormalityat occurs in about 40 to 50 of every 100 000 live births, with2:1 ratio in males versus females. Most lesions are treatedon after birth or in childhood, and adults presenting withtreated coarctation of the aorta are rare. More often,tients have had previous procedures and present with lateroblems such as heart failure, intracranial hemorrhage,pertension particularly with exercise, aneurysm formation,

oD, rupture of old repairs, undersized grafts of previouspairs, and infections. It is of particular importance ineviously treated patients with some aortic narrowing toeck for a gradient across the stenosis and for hypertensionring exercise testing (154).Untreated coarctation of the aorta has a dismal prognosis,

ith 80% of patients dying from complications associatedith the coarctation. Approximately one quarter will die fromoD or rupture, one quarter will die from heart failure, onearter will die from intracranial hemorrhage, and the re-
ainder will die from other complications.
Surgical options, depending on the lesion, include subcla-an artery patch aortoplasty, patch aortoplasty, bypass of thearctation, tube graft replacement, aneurysm replacement,stage combined bicuspid valve surgery, and arch andscending aorta replacement or ascending aorta–to–de-ending aorta bypass (3). Endovascular balloon dilatationd stent placement has been used successfully and iscoming a less invasive alternative to conventional openrgical procedures (155). Occasionally, the adult aorta mayredundant and kinked opposite the ligamentum arteriosum

ithout any pressure gradient, the so-called pseudocoarcta-n. Aneurysms that require surgical treatment may developoximal and distal to the kinked area (156).

.4. Right Aortic Archright-sided aortic arch is present in approximately 0.5% ofe population and rarely requires surgical repair. However,me patients present with dysphagia or asthma-like symp-ms with expiratory wheezing. CT or MR readily diagnosese problem of either tracheal compression or esophagealmpression with the esophagus enlarged and filled with gasove the level of the arch. Felson and Palayew described 2pes (157).In Type I, the great vessels come off the right-sided arch in

manner that is a mirror image to normal anatomy; however,mpression of the esophagus or trachea is caused by anlarged aorta where it crosses the vertebral bodies or by thescular ring formed by the atretic ductus arteriosus. In Type

, the aberrant left subclavian artery comes off the descend-g aorta and typically runs posterior to the trachea andmpresses the trachea. As with an aberrant right subclaviantery, the proximal segment of the subclavian artery maylarge and form a Kommerell diverticulum. Other variationse also seen but are exceedingly rare. A separate trunk mayise from the arch, and from this trunk, the innominate,rotid, and subclavian arteries arise. Another rare finding isright-sided arch with a stump of left-sided aorta joining theght-sided arch after it crosses into the left chest. The stumpthe left-sided aorta gives off a branch to the left subclavian

tery. The aorta with right-sided arches is also abnormal andpically very fragile and prone to AoD, rupture, or aneurysmrmation. Surgical repair involves resection of the aorta and,needed, reimplantation or bypass of the aberrant left

bclavian artery.

. Inflammatory Diseases Associatedith Thoracic Aortic Disease

.1. Recommendations for Takayasu Arteritisnd Giant Cell Arteritise Table 8.

ASS I

Initial therapy for active Takayasu arteritis and active giantcell arteritis should be corticosteroids at a high dose (pred-nisone 40 to 60 mg daily at initiation or its equivalent) toreduce the active inflammatory state. (158,159) (Level of
Evidence: B)

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The success of treatment of patients with Takayasu arteritisand giant cell arteritis should be periodically evaluated todetermine disease activity by repeated physical examinationand either an erythrocyte sedimentation rate or C-reactiveprotein level. (160,161) (Level of Evidence: B)Elective revascularization of patients with Takayasu arteritisand giant cell arteritis should be delayed until the acuteinflammatory state is treated and quiescent. (162) (Level ofEvidence: B)The initial evaluation of Takayasu arteritis or giant cell arteritisshould include thoracic aorta and branch vessel computedtomographic imaging or magnetic resonance imaging to inves-tigate the possibility of aneurysm or occlusive disease in thesevessels. (Level of Evidence: C)

ASS IIa

It is reasonable to treat patients with Takayasu arteritisreceiving corticosteroids with an additional anti-inflammatoryagent if there is evidence of progression of vascular disease,recurrence of constitutional symptoms, or re-elevation of in-flammatory marker. (158) (Level of Evidence: C)

.2. Takayasu Arteritisakayasu arteritis, also known as pulseless disease, is aniopathic vasculitis of the elastic arteries, involving the aortad its branches. Initially described in Japan, the disease is

ble 8. Inflammatory Diseases Associated With Thoracicrtic Aneurysm and Dissection

mesCriteria Used in

Diagnosis/SourceWhen Is Diagnosis

Established?

kayasueritis (163)

Age of onset �40 y �3 criteria arepresent (sensitivity90.5%; specificity

97.8%)

Intermittent claudication

Diminished brachial arterypulse

Subclavian artery or aortic bruit

Systolic BP variation of�10 mm Hg between arms

Aortographic evidence of aortaor aortic branch stenosis

nt celleritis (164)

Age �50 y �3 criteria arepresent (sensitivitygreater than 90%;specificity �90%)

Recent-onset localizedheadache

Temporary artery tenderness orpulse attenuation

Elevated erythrocytesedimentation �50 mm/h

Arterial biopsy showsnecrotizing vasculitis

hcetease (165)

Oral ulceration Oral ulceration plus 2of the other 3 criteriaRecurrent genital ulceration

Uveitis or retinal vasculits

Skin lesions—erythemanodosum, pseudo-folliculitis, or

pathergy

kylosingondylitis (166)

Onset of pain �40 y 4 of the diagnosticcriteria are presentBack pain for �3 mo

Morning stiffness

Subtle symptom onset

Improvement with exercise

BP indicates blood pressure.

und worldwide. In the United States, a review of cases in ry

lmsted County, Minn, reported a rate of 2.6 cases perillion persons (158). In the United States, the disease affectsl ethnic and racial groups in proportion to the census with aoderate Asian overrepresentation. The disease affectsomen approximately 10 times more often than men. Mostmmonly diagnosed in the third decade (i.e., the 20s) of life,e disease has been found in children and adults in the fifthcade (i.e., the 40s). Two specific disease distributions haveen reported: Japanese and Indian (167,168).In the Japanese distribution, the thoracic aorta and greatssels are most commonly affected. In contrast, in the Indianpe, the disease most commonly affects the abdominal aortad the renal arteries (28). The pathogenesis of Takayasuteritis remains poorly defined. A T-cell–mediated panarteri-, the disease proceeds from adventitial vasa vasorumvolvement inward. The antigen for the localized inflamma-ry process is undefined but likely specific as the T cellsdergo clonal expansion. The outcome process of destruc-n and fibrotic repair depends on the dominant pathophys-

logic process: destruction yields aneurysms while fibrosisuses stenosis.The diagnosis of Takayasu arteritis may be made using the90 American College of Rheumatology criteria: 1) age ofset younger than 40 years, 2) intermittent claudication, 3)minished brachial artery pulse, 4) subclavian artery orrtic bruit, 5) systolic blood pressure variation of greateran 10 mm Hg between arms, and 6) angiographic (CT, MR)idence of aorta or aortic branch vessel stenosis (163)igure 16). When 3 of the criteria are manifest, the sensi-ity and specificity for diagnosis are 90.5% and 97.8%,

spectively. Laboratory testing may aid in diagnosis. Mark-s of inflammation, such as C-reactive protein and erythro-te sedimentation rate, are elevated in approximately 70% oftients in the acute phase and 50% in the chronic phase ofsease (158).The clinical manifestations of the disease typically develop2 phases: acute and chronic. Acutely, the inflammation

sociated with Takayasu arteritis causes a host of constitu-nal, or “B,” symptoms, such as weight loss, fatigue, nighteats, anorexia, and malaise (158). More chronically, once

e vascular process has endured, patients report symptomsferable to the organs involved. In the largest US experience,ore than half of all patients experienced upper extremityaudication, half had symptoms associated with cerebrovas-lar insufficiency (vision loss, lightheadedness, stroke), andthird reported carotid artery pain (158). In an Indian series,pertension as a result of renal artery involvement was theost common presenting sign (169).The aorta itself may develop either aneurysm or stenosis.a Japanese series of 116 patients with Takayasu arteritis,arly 32% of the patients had aortic aneurysm formation70). Most commonly, aneurysm formation developed in thescending aorta, followed by the abdominal, then ascendingrtic segments. In the National Institutes of Health series ofpatients with Takayasu arteritis, 23% had aortic aneurysm

rmation (158). Aneurysms most commonly formed in thertic arch or root, abdomen, and then other thoracic seg-ents. Stenosis of the aorta is more common than is aneu-
sm formation, occurring in 53% of patients in the National

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gure 16. Takayasu arteritis with involvement of the thoracoabdominal aorta and great vessels as shown on contrast-enhanced CTd MR examinations. Note narrowing of the arterial lumen and circumferential soft tissue thickening of the walls of the great vesselsd thoracic and abdominal aorta. Panel A, Image through the great vessels with narrowing of the left common carotid and left subcla-

an arteries. Panel B, Mid descending thoracic aorta (arrowheads). Panel C, Aorta just above the diaphragm (arrowheads). Panel D,frarenal aorta. Panel E, Volume-rendered image from CT demonstrates the extent of involvement. Panel F, Oblique sagittal MR of theoracic aorta. Panel G, Coronal MR of the abdominal aorta. CT indicates computed tomographic imaging; and MR, magnetic reso-
nce imaging.

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stitutes of Health series. Any segment of the aorta may bevolved, but the abdominal aortic segment is affected morean 70% of the time if stenosis is found.Treatment of Takayasu arteritis begins with inflammationduction with corticosteroids. Steroids are typically started atgh dose, 40 to 60 mg daily at initiation to lower theythrocyte sedimentation rate or C-reactive protein to nor-al, and are required for 1 to 2 years to ensure proper diseaseeatment (169). Despite the prolonged regimen, nearly half

the patients will relapse during tapering, requiring addi-nal immunosuppression. Second-line agents that have beened include methotrexate, azathioprine, and antitumor ne-osis factor-alpha agents (158,169). Unfortunately, markersinflammation are imperfect barometers of disease activity.

isease progression has been shown to occur in the setting ofrmal marker levels (158).Revascularization for aortic stenosis or aneurysm occursr the same indications as in noninflammatory disorders:condary organ vascular insufficiency or risk of rupture.here are no randomized trials of percutaneous or surgicaltervention in this disease (158,171–173). Nonrandomizedports have shown that revascularization of either varietyay be appropriate, with one caveat. The risk of graft failurehigher in patients with active local inflammation (162).oreover, the presence of aneurysmal disease itself may beoblematic. One report documented a 12% incidence ofastomotic aneurysms over 2 decades of follow-up related toe presence of aneurysms at surgery (174).

.3. Giant Cell ArteritisCA, also known as temporal arteritis, is an elastic vesselsculitis involving the aorta and its secondary and tertiaryanches. Distinguishing it from Takayasu arteritis, GCAfects patients above the age of 50 years, with an incidenceaking in the eighth decade of life (175). The disease affectsomen in a 3:2 ratio to men and has a predilection for those

northern European ancestry (29). In the United States,idemiologic investigation reports a prevalence of 20 casesr 100 000 persons. The incidence is higher in Scandinaviantions but lower in southern Europe, suggesting a geneticedisposition in certain populations (176).The clinical presentation of GCA is varied, requiring aightened suspicion by clinicians for early diagnosis. Half ofe patients report constitutional symptoms, such as weightss, night sweats, malaise, and fever (177). Because of theedilection for secondary and tertiary thoracic branches ofe aorta, cranial symptoms are common. Scalp tendernessd headache are present in two thirds of patients and in up90% of patients with biopsy-proved disease (177). Jaw

audication is common and affects half of the patients, 20%velop visual changes, and other neurologic symptoms suchstroke or neuropathy occur in nearly one third (29). Visualanges are particularly important to notice, because early

eatment may prevent permanent blindness. Patients mayport diplopia, amaurosis fugax, or blurriness prior to blind-ss. Polymyalgia rheumatica characterized by a generalizedflammatory state with proximal muscle involvement is
und in nearly half of patients with GCA (29). Patients with ar
lymyalgia rheumatica report muscular pain and stiffness,rticularly on initiation of movement.Extracranial vascular involvement is less common in GCAan in Takayasu arteritis, occurring in 25% of patients. In a-year study of Olmsted County, Minn, that included 168tients with GCA, aortic aneurysm/dissection was found in% of the subjects, whereas large-artery stenosis was noted13% of patients (178). No patient had stenosis of the aorta.

ortic aneurysm formation represents an important marker.lthough aneurysm formation per se does not reduce survivalmpared with the GCA cohort as a whole, AoD in the settingan aneurysm reduces survival to an average of 1.1 years

78). Similarly, aortic aneurysm rupture or dissection causedo thirds of deaths in a series of patients with GCA in

alifornia (179).The American College of Rheumatology diagnostic criteriar GCA include 1) age older than 50 years, 2) recent-onsetcalized headache, 3) temporal artery pulse attenuation ornderness, 4) erythrocyte sedimentation rate greater than 50m/h, and 5) an arterial biopsy demonstrating necrotizingsculitis (164). Three or more criteria confer a sensitivityd specificity above 90% for the disease. With intracranialsease, temporal artery biopsies are diagnostic in up to 80%cases (180). The rate of positivity declines with initiationglucocorticoid therapy, but this should not delay treatmentavoid GCA complications. Biopsies performed within 7

ys of steroid initiation retain a high diagnostic yield (181).The pathophysiology of GCA shares important features

ith Takayasu arteritis (28). GCA is marked by a T-cellonal expansion suggesting a specific antigenic response,hich currently remains unelucidated. The inflammatorysponse, which begins in the adventitial layer, is marked bygmented cytokine and MMP production causing granulomarmation. Granuloma formation both shields the vessel frome inciting antigen and causes vessel destruction. The in-mmatory environment within the vessel wall with thessible formation of aneurysms or vessel stenosis is histo-gically identical to that of Takayasu arteritis. Because of theultiyear cyclical nature of disease incidence, some havesited an infectious etiology (29).Corticosteroids represent the standard in therapy for pa-nts with GCA (182). The typical treatment regimen in-

udes starting prednisone dose of 40 to 60 mg daily, althoughcent evidence suggests a similar efficacy with 30 to 40 mgily (183). Therapy is typically required for 1 to 2 years tooid recurrence, although the dose may be tapered beginningto 3 months after initiation. Patients commonly report

eling much better rapidly but, as with Takayasu arteritis,w vascular involvement may occur in up to half of patients

eated with steroids (184). In contrast to Takayasu arteritis,ditional immunomodulatory agents do not seem to modu-te the disease’s progress. Methotrexate studied in a double-ind, placebo-controlled study as an adjunct to prednisoned not reduce morbidity, erythrocyte sedimentation ratevel, or cumulative prednisone dose (184a). Revasculariza-n recommendations follow the same pattern as in Takayasu

teritis.

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.4. Behcet Disease1937, Hulusi Behçet described his eponymous syndrome

sed on a set of 3 symptoms: uveitis, aphthous stomatitis,d genital ulcers. Most common in Turkey, with a preva-nce of 80 to 370 cases per 100 000 persons (185,186), thesease is much less common in the United States, with antimated prevalence of 1 to 3 cases per million persons87). The diagnostic criteria were established by the Inter-tional Group for Behçet’s disease and require oral ulcer-ion and 2 of these 3 lesions: recurrent genital ulceration,eitis or retinal vasculitis, or skin lesions, such as erythemadosum, pseudofolliculitis, or pathergy (188). In addition toese cardinal manifestations, vascular involvement maycur in one third of patients. A small vessel vasculitismmonly associated with the human leukocyte antigenLA) B51 allele (187), Behçet disease is 1 of 2 vasculitides

at may also involve veins. Venous involvement is mostmmonly superficial thrombophlebitis, but deep vein throm-sis in the vena cava, varices, and cerebral sinuses has beenported (189). The small vessel involvement of Behçetsease may result in nonvascular complaints, such as ery-ema nodosum, arthritis, and gastrointestinal involvementith diarrhea, gastrointestinal bleeding, or perforation (187).reatment of Behçet disease varies based on the manifesta-n of disease. Systemic corticosteroids are the typical

erapy for those with vascular involvement.Specifically with regard to vascular manifestations of

ehçet disease, any artery or vein, large or small, systemic orlmonary, may be involved by the vasculitic process. Aorticstopathology shows lymphocytic infiltration mixed withstiocytes and eosinophils with giant cells around vasasorum of media and adventitia. Destruction of media leadsaneurysm formation and may proceed to pseudoaneurysm

rmation and rupture. Aneurysm formation may occur inultiple sites and in different sites over a period of follow-up.neurysm, stenotic lesions, and occlusion of brachiocephalicteries may occur with or without aortic involvement.lthough aortic involvement is unusual for patients withehçet vasculitis, aneurysm rupture can be unpredictable andtal (190,191). With regard to surgical repair, anastomoticeudoaneurysms often occur (12.9% within 18 months in 1ries) and may be related to ongoing inflammatory changesthe area of anastomotic suture lines (192). Endovascular

pair with stent grafts has also been described (193).

.5. Ankylosing Spondylitispondyloarthropathies)

he group of diseases labeled spondyloarthropathies areked by the strong association of major histocompatibilitymplex HLA B-27 and the absence of rheumatoid factor94,195). Several features are common to the spondyloar-ropathies, including sacroilitis, inflammatory arthritis orthesitis (inflammation of tendon insertions), associationsith inflammatory bowel disease or psoriasis, and aortitis andart block (195).Ankylosing spondylits is the most common variant andten begins with back pain and stiffness in the second or
ird decade of life. It affects men 2 to 3 times as often as cr
omen, worsens with inactivity, and commonly takes yearsr the diagnosis to be made (196). The diagnosis requires 4the 5 criteria: onset of pain at younger than 40 years, backin for longer than 3 months, morning stiffness, subtlemptom onset, and improvement with exercise (197). Pa-nts may also report constitutional symptoms, such asalaise or fever. Acute anterior uveitis is reported in up to% of patients. Aortic root and aortic valve involvement areported in up to 80% of patients (198). When involved, thertic valve may have a nodular appearance, and aorticlvular regurgitation is present in nearly half of the patients98). Treatment of aortic root expansion and aortic valvularnormalities is the same as for other conditions.

.6. Infective Thoracic Aortic Aneurysmsfection (due to bacterial, fungal, viral, spirochetal, orbercular organisms) is a rare cause of thoracic aorticeurysms. Originally named mycotic endarteritis by Osler99), the terms infected aneurysm or infectious aortitis arew used more commonly, because the majority of etiologicents are nonfungal. Saccular aneurysms are most common,t infected aneurysms can be fusiform and often eveneudoaneurysms. The ascending thoracic aorta, aortic arch,d descending thoracic aorta can all be affected, as canosthetic aortic grafts and aortic homografts. Typically, thetes of infected aneurysms are opposite the great vessels ine aortic arch or opposite the visceral ateries in the abdomen.here are several mechanisms by which the aortic infectionay arise. First, there may be contiguous spread fromjacent thoracic structures, such a mediastinitis, abscess,fected lymph nodes, infectious pericarditis, empyema, orravertebral abscess. Second, there may be septic emboli

om underlying bacterial endocarditis. Third, there may bematogenous dissemination of bacteria in the setting ofpsis or intravenous drug abuse. Infection most often arisesa diseased aorta, either in a preexisting aneurysm, at the

te of an atherosclerotic plaque, or at the site of somecidental or iatrogenic aortic trauma. Indeed, infected tho-cic aortic aneurysms may arise as a late complication ofrdiac surgery, often associated with postoperative medias-itis, typically at the sites of aortic cannulation or anasto-

otic suture lines (200,201).Various organisms can infect the aorta, with most infec-ns being bacterial. Staphylococcus aureus and Salmonella

e the organisms most commonly identified (202–204).neumococcus and Escherichia coli are relatively commonam-positive and gram-negative pathogens, respectively.Treponema pallidum, the gram-negative spirochete bacte-

um that causes syphilis, as well as other Treponema species,n cause infected aortitis, with the ascending thoracic aortaost often involved. However, in syphilitic aortitis, thoracicrtic aneurysm does not appear for 10 to 25 years after theitial spirochetal infection. Fungal infections of the aorta,ith either Candida or Aspergillus, occur less often (205) andpically occur in the setting of impaired immunity, such astients with systemic illness, human immunodeficiencyrus, or prior organ or bone marrow transplant.Indeed, patients with impaired immunity are also at in-
eased risk of tuberculous aortitis attributable to mycobac-

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rium tuberculosis. Tuberculous aortitis has until now beenceedingly rare, but the incidence may rise as the prevalencetuberculosis rises worldwide. Tuberculous aortitis typically

fects the distal aortic arch and descending thoracic aorta,ely because the aorta is thought to become infected via

rect extension from continuous infected lymph nodes,pyema, or pericarditis (206).Finally, there appears to be an independent associationtween human immunodeficiency virus and ascending tho-cic aortic dilatation, although its mechanisms are poorlyderstood (207,208). Moreover, the incidence of frankeurysms remains extremely low.

. Acute Aortic Syndromes

cute aortic syndromes consist of 3 interrelated conditionsith similar clinical characteristics and include AoD, IMH,d PAU (209).

.1. Aortic Dissection

1.1. Aortic Dissection DefinitionoD is defined as disruption of the media layer of the aortaith bleeding within and along the wall of the aorta resultingseparation of the layers of the aorta. In the majority of

tients (90%), an intimal disruption is present that results inacking of the blood in a dissection plane within the media.his may rupture through the adventitia or back through thetima into the aortic lumen (Figure 17). This classic dissec-n results in a septum, or “flap,” between the 2 lumensigure 18). The false lumen may thrombose over timeigure 19). While on noninvasive imaging, 15% of patientsith aortic dissection syndromes have an apparent IMHithout evidence of an intimal tear, autopsy studies showly 4% have no visible intimal tear; indeed, at the time ofrgery a tear is found in most patients (210,211). Occasion-ly, AoD originates from a small atheromatous ulcer that isfficult to identify. On the other hand, extensive atheroma-us disease of the aorta may lead to PAU or a localized IMH.The true incidence of acute AoD is difficult to define for 2incipal reasons: 1) acute AoD can be rapidly fatal, andhen patients die prior to hospitalization, death may beroneously attributed to another cause and 2) acute AoD isequently missed on initial presentation, and early mortality

ong this group may be misclassified as non–dissectionlated. Population-based studies suggest that the incidence ofute AoD ranges from 2 to 3.5 cases per 100 000 person-ars, which correlates with 6000 to 10 000 cases annually ine United States (75,212,215–217). A review of 464 patientsom IRAD reported a mean age at presentation of 63 years,ith significant male predominance (65%) (47). The preva-nce of AoD appears to be increasing, independent of theing population, as noted by Olsson and colleagues (218),ho found the incidence of AoD among Swedish men hascreased to 16 per 100 000 men yearly. It may be that 2 totimes as many patients die from AoD than from rupturedAA; approximately 75% of patients with AAA will reach anergency department alive, whereas for AoD, the prognosis

pears to be worse, with 40% dying immediately, 1% per de

ur dying thereafter, and between 5% and 20% dying duringshortly after surgery (219–221). Furthermore, only 50% to% will be alive 5 years after surgery depending on age andderlying etiology (222). Because AoD tends to occur ineas of aneurysmal dilatation, treatment of aneurysms beforessection occurs is important to long-term survival (3) (seection 8.1).Regarding time from onset of initial symptoms to time ofesentation, acute dissection is defined as occurring within 2eeks of onset of pain; subacute, between 2 and 6 weeksom onset of pain; and chronic, more than 6 weeks fromset of pain.

1.2. Anatomic Classification of Aortic Dissectionnatomically, acute thoracic AoD can be classified according

either the origin of the intimal tear or whether thessection involves the ascending aorta (regardless of the site

origin). Accurate classification is important as it drives

gure 17. Classes of intimal tears. I. Classic dissection withtimal tear and double lumen separated by septum. Communi-tion between lumens is typically in descending aorta ateared-off intercostal arteries or distal reentry site. II. IMH. Notimal tear or septum is imaged but is usually found at surgeryautopsy. DeBakey Types II and IIIa are common extent of this

sion. III. Intimal tear without medial hematoma (limited dissec-n) and eccentric aortic wall bulge. Rare and difficult to detectTEE or CT. Patients with Marfan syndrome prone to this

pe. May result in aortic rupture or extravasation. IV. PAU usu-ly to the adventitia with localized hematoma or saccular aneu-sm. May propagate to Class I dissection, particularly whenvolving ascending aorta or aortic arch. V. Iatrogenic (cathetergiography or intervention)/traumatic (deceleration) dissection.

T indicates computed tomographic imaging; IMH, intramuralmatoma; PAU, penetrating atherosclerotic ulcer; and TEE,nsesophageal echocardiography. Figure reprinted with per-

ission from the Cleveland Clinic Foundation. Legend adaptedm Svensson et al (212), Chirillo et al (213), and Murray et al

14).

cisions regarding surgical versus nonsurgical management.

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he 2 most commonly used classification schemes are theeBakey and the Stanford systems (Figure 20). For purposes

classification, the ascending aorta refers to the aortaoximal to the brachiocephalic artery, and the descendingrta refers to the aorta distal to the left subclavian artery.The DeBakey classification system categorizes dissectionssed on the origin of the intimal tear and the extent of thessection:

Type I: Dissection originates in the ascending aorta andpropagates distally to include at least the aortic arch and

gure 18. Type A aortic dissection and extent of involvement depough the flap appears to disappear in the infrarenal, it is actuallyads) and it is clearly present caudally in the common iliac arterie

all thickening (arrowheads) indicates ischemia in Panel I. Panel Aove the diaphgram. Panel E, At the level of the celiac axis. Paneon iliac arteries. Panel I, Image through the mid abdomen at narre to bowel ischemia caused by apposition of the flap against th

s. CT indicates computed tomographic imaging; F, false lumen; a

typically the descending aorta (surgery usually recommended). th

Type II: Dissection originates in and is confined to theascending aorta (surgery usually recommended).Type III: Dissection originates in the descending aorta andpropagates most often distally (nonsurgical treatment usu-ally recommended).– Type IIIa: Limited to the descending thoracic aorta.– Type IIIb: Extending below the diaphragm.

The Stanford classification system divides dissections intocategories, those that involve the ascending aorta and those

n axial CT images from the cranial to caudal direction. Al-ressed against the anterior wall of the aorta in Panel G (arrow-nel H. Hemopericardium (asterisk) is visible in Panel D. Bowelarch. Panel B, Mid thorax. Panel C, Aortic root. Panel D, Just

d kidneys. Panel G, Infrarenal aorta. Panel H, Proximal com-dow/level settings demonstrates small bowel wall thickenings of the celiac axis and superior and inferior mesenteric arter-true lumen.

icted ocomps in Pa, Aorticl F, Miow wine origin

at do not.

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Type A: All dissections involving the ascending aortaregardless of the site of origin (surgery usually recom-mended) (Figures 18 and 20).

Type B: All dissections that do not involve the ascendingaorta (nonsurgical treatment usually recommended). Noteinvolvement of the aortic arch without involvement of the

Figure 19. Type A Aortic dissection with throm-bosed false lumen and left renal artery involvementdepicted on axial CT images. Demonstratesmarked narrowing of the true lumen, patent rightrenal artery arising from the true lumen (bottomleft, arrow), and narrow left renal artery com-pressed by thrombus in the false lumen, with sec-ondary decreased enhancement of the left kidneycompared with the right kidney. Top left, At thelevel of the left main coronary artery. Top right, Atthe celiac axis. Bottom left, At the right renal artery(arrow). Bottom right, At the left renal artery (ar-row). *Thrombus in false lumen. CT indicates com-puted tomographic imaging; L, left kidney; and R,right kidney.

Figure 20. Aortic dissection classification:DeBakey and Stanford Classifications. Re-printed with permission from the ClevelandClinic Foundation.

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ascending aorta in the Stanford classification is labeled asType B (Figure 21).

At this time, there is no unanimity regarding whichassification system is the ideal one to use. Some of theriting committee members believe that a more pragmaticproach is to refer to the dissection involving the aorta asther proximal or distal to the left subclavian artery. Othersthe writing committee do not use this approach. Thus, if atient has an arch dissection even without ascending aorticvolvement, then immediate surgery would be recommended

some, if feasible and the patient is viable. Others on theriting committee would select medical management if thetient has only an arch dissection without proximal exten-

on, malperfusion, or bleeding, as long as repeat imagingmonstrates stability. If there is evidence of malperfusion oreeding in such a patient, then the writing committee wouldually select a surgical approach.The intimal tear and AoD can also be categorized into

asses that may have a bearing on treatment (212,213)igure 17).

1.3. Risk Factors for Aortic Dissectionisk factors for AoD include conditions that result in aorticedial degeneration or place extreme stress on the aortic wallable 9). Two thirds to three quarters of patients havepertension, which is often uncontrolled. Genetic predispo-

tion (see Section 5) to AoD can occur in the context of androme, such as Marfan syndrome or Loeys-Dietz syn-ome, or can be inherited in families in the absence ofndromic features (3). IRAD data showed that of patientsder 40 years of age with AoD, 50% had a history of Marfanndrome (223). Other congenital or genetically based dis-ses as well as inflammatory conditions associated with agher risk of AoD are noted in Sections 6.3, 6.4, and 7.First and foremost, a family history of thoracic aorticeurysm is an important risk factor. In 2 separate clinical

udies, 13% to 19% of patients without an identified geneticndrome with thoracic aortic aneurysms had first-degreelatives with thoracic aortic aneurysms or AoD (127,130).he term “familial thoracic aortic aneurysm and dissectionndrome” is often applied (see Section 5). In taking a historyr thoracic aortic disease, one should be careful to distin-ish a history of an abdominal aortic aneurysm from aoracic aortic aneurysm. Many people, even healthcare
oviders, mistakenly use the terms AAA or triple A for any
rtic aneurysm, regardless of location. Clarifying that theeurysm was thoracic rather than abdominal affects one’snsideration of risk. Also, one must consider the potentialderlying diagnosis when a patient reports a family history“sudden death” or “heart attack” when there was no

nfirmatory autopsy. If the patient’s father, at the age of 45,d sudden onset of chest pain and then died moments later,ere is a chance that the death may have been from an acuteoD rather than an acute MI.The history may reveal syndromic causes of thoracic aorticeurysm and dissections, especially Marfan, Loeys-Dietz,d vascular Ehlers-Danlos syndromes. In some cases, pa-nts have only some of the features of Marfan or Loeys-

ietz syndrome, rather than the full-blown clinical syndrome,

Figure 21. Type B aortic dissection with mediasti-nal hematoma and pleural blood. Ruptured Type Baortic dissection with mediastinal hematoma (*)and pleural blood. Left, Flap arises in the proximaldescending thoracic aorta, with faint contrast-en-hanced blood adjacent to the site of rupture out-side the confines of the aortic wall (arrow). Right,At the level of the aortopulmonary window. FL in-dicates false lumen; PL, pleural blood; and TL, truelumen.

ble 9. Risk Factors for Development of Thoracicrtic Dissection

nditions associated with increased aortic wall stress

Hypertension, particularly if uncontrolled

Pheochromocytoma

Cocaine or other stimulant use

Weight lifting or other Valsalva maneuver

Trauma

Deceleration or torsional injury (e.g., motor vehicle crash, fall)

Coarctation of the aorta

nditions associated with aortic media abnormalities

Genetic

Marfan syndrome

Ehlers-Danlos syndrome, vascular form

Bicuspid aortic valve (including prior aortic valve replacement)

Turner syndrome

Loeys-Dietz syndrome

Familial thoracic aortic aneurysm and dissection syndrome

Inflammatory vasculitides

Takayasu arteritis

Giant cell arteritis

Behcet arteritis

Other

Pregnancy

Polycystic kidney disease

Chronic corticosteroid or immunosuppression agent administration

Infections involving the aortic wall either from bacteremia or extensionof adjacent infection

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a history of any phenotypic features, such as mitral valveolapse or pectus excavatum, should prompt considerationthoracic aortic aneurysms or dissections (224,225). Bicus-

d aortic valve is a strong risk factor for ascending thoracicrtic aneurysms, as well as coarctation of the aorta. Indition, a history of extreme exertion or emotional distressay preceed the onset of pain (226).

1.4. Clinical Presentation of Acute Thoracicortic Dissectionhe clinical presentation of acute AoD spans a spectrum frome overt with classic pain and physical examination findings

the enigmatic as a painless process with few physicalanifestations of the disease (Table 10). Given its exceed-gly high mortality, clinicians must maintain a high index ofspicion for acute AoD, as noted in Section 8.6 (Figure 22).

1.4.1. SYMPTOMS OF ACUTE THORACIC

RTIC DISSECTION

tients with acute aortic syndromes often present in a similarshion, regardless of whether the underlying condition isoD, IMH, PAU, or contained aortic rupture. Pain is the mostmmonly reported presenting symptom of acute AoD re-rdless of patient age, sex, or other associated clinicalmplaint (228–235). Pooled data from over 1000 patients instudies found that the pain of acute dissection is perceivedabrupt in onset in 84% of cases (95% CI 80% to 89%) andsevere intensity in 90% of cases (95% CI 88% to 92%)

36). Although classically described as having a tearing orpping quality, registry data suggest patients are more likely

describe the pain of acute dissection as sharp or stabbing1% to 64%, respectively) and that report of a migratingality to pain is highly variable (12% to 55%) (228,236).in may subsequently ease or abate, leading to a false

ble 10. International Registry of Acute Aortic DissectionAD) Physical Findings of 591 Patients With Type Artic Dissection

esenting Hemodynamics and Clinical Findings Frequency/Finding

pertensive 32%

rmotensive 45%

potensive 14%

ock 13%

rdiac tamponade 5%

urmur of aortic insufficiency 45%

lse deficits 26%

ricardial friction rub 2%

rebrovascular accident 8%

hemic peripheral neuropathy 3%

hemic spinal cord damage 2%

hemic lower extremity 10%

ma/altered consciousness 12%

ngestive heart failure 5%

st blood pressure systolic, mean 130 mm Hg

st blood pressure diastolic, mean 75 mm Hg

Adapted from Pape et al (227).

assurance on the part of the patients and physicians. th

Pain location and other associated symptoms reflect thete of initial intimal disruption and may change as thessection extends along the aorta or involves other arteries organ systems (236). Data from 464 patients enrolled inAD found that patients with Type A dissections most

equently present with chest pain (80%), more commonlyscribed as anterior (71%) than as posterior (32%) (228).lthough less common, patients with Type A dissectionport back pain (47%) and abdominal pain (21%), presum-ly as a result of antegrade dissection into the descendingrta (228). In contrast, patients with Type B dissections areost likely to present with back pain (64%) followed by chestd abdominal pain (63% and 43%, respectively) (228).me patients present with abdominal pain in the absence ofest pain or with only painful or numb lower extremitieslated to end-organ ischemia. In 1 retrospective study of 44tients ultimately diagnosed with acute thoracic AoD, thecation of the patient’s pain was highly predictive of theinician’s suspicion for acute AoD; dissection was suspected86% of patients who presented with chest and back pain,% of those who presented with chest pain alone, and only

of those primarily abdominal pain (237).

Although uncommon, acute AoD may present without pain38–240). In a separate analysis of 977 IRAD patients, 63tients (6.4%) presented without pain (241,242). This grouppatients was noted to be older and more likely to present

ith syncope, stroke, or congestive heart failure than weretients with painful dissection (241). Patients on steroids andtients with Marfan syndrome may be more prone to presentithout pain (243).

1.4.2. PERFUSION DEFICITS AND END-ORGAN ISCHEMIA

rfusion deficits as a result of dissection-related obstructionaortic branch vessels have long been recognized as a

mmon clinical manifestation, resulting in organ complica-ns at initial presentation (Table 11). End-organ involve-

ent in acute thoracic AoD can occur via several mecha-sms. Most occlusions are caused by obstruction by thessection flap, which can either prolapse across a vesseligin without entering it (dynamic obstruction) or directlytend into a vessel (static obstruction) (244) (Figure 18).ther causes include postobstructive arterial thrombosis,bolism to branches of either the true or false lumen, direct

mpression of an aortic branch artery or adjacent structuresan expanding false lumen (245), rupture or leakage of the

lse lumen into contiguous structures, and occlusion orssection of coronary arteries and/or aortic valve distortionading to heart failure.

Physical examination is insensitive to renal and mesentericchemia early in the course of acute AoD. Elevated serumeatinine or refractory hypertension may be due to renalchemia but may represent the clinical baseline in a patientith poorly documented or inadequately treated prior medicalnditions. Serologic markers of mesenteric ischemia mayt be present until hours after onset.

Combined data from over 1500 patients in 16 studies found
at pulse deficits were present in 31% of cases (95% CI 24%

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39%) and, when present, were strongly suggestive of AoDositive likelihood ratio 5.7; 95% CI 1.4 to 23) (37) andedict increased risk. Of 513 cases of Type A dissection,tients with perfusion deficits were more likely to presentith hypotension, shock, neurologic deficits, and tamponaded were more likely to have higher rates of hospitalmplications and mortality (41% versus 25%, P�0.0002)46). Furthermore, overall mortality rates correlated with the

gure 22. Acute surgical management pathway for AoD. *Additionssection; CABG, coronary artery bypass graft surgery; CAD, coroophageal echocardiogram.

mber of pulse deficits present, likely as a reflection of the pa

tent of vascular compromise and associated end-organchemia (246). Similarly, of 118 patients with Type A acutessection, limb ischemia (defined as loss of pulse withsociated pain and neurologic symptoms) was present in 38ses (32%) (247). The presence of limb ischemia wassociated with an increased likelihood of other end-organchemia (i.e., cerebral, visceral, or coronary) and a signifi-nt increase in overall mortality (247). Among the 38

appropriate’ based on Patel et al. (226a) AoD indicates aorticrtery disease; TAD, thoracic aortic disease; and TEE, trans-

of ‘ifnary a

tients with limb ischemia, in-hospital mortality was 45%

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mpared with 15% among the 61 patients without organalperfusion (247).

These studies underscore the clinical importance of anequate vascular examination to help both identify thesease and stratify risk once the diagnosis is established.very patient being evaluated for possible acute AoD shouldve pulses checked in all extremities to identify the presenceperfusion deficits. In patients with acute limb ischemia

rsus those without, renal and mesenteric malperfusion werearly 2-fold more frequent and mortality was twice as high,rther highlighting the importance of this finding (248).

1.5. Cardiac Complicationshe heart is the most frequently involved end organ in acuteoD involving the ascending aorta. In distinction to otherd-organ pathology, most cardiac complications are a directsult of dissection-related disruption of normal anatomiclationships (215,245).

1.5.1. ACUTE AORTIC REGURGITATION

cute aortic regurgitation is the most commonly recognizedrdiac complication of Type A dissection (228–234), occur-

ng in 41% to 76% of cases (228–232). Three distinctssection-related mechanisms for acute aortic valve incom-tence have been identified, and they can occur in combi-tion: 1) acute dilatation of the aortic root by an expandinglse lumen, resulting in incomplete aortic valve closure; 2) assection extending into the aortic root and disrupting aorticlve commissural attachments, resulting in valve leafletolapse; and 3) a portion of dissection flap prolaping throughe aortic valve in diastole, preventing adequate leafletosure (235). Clinical manifestations of dissection-relatedrtic regurgitation span the spectrum from only a hemody-mically insignificant diastolic murmur to congestive heartilure and cardiogenic shock (236,249).

1.5.2. MYOCARDIAL ISCHEMIA OR INFARCTION

yocardial ischemia or infarction is an infrequent but serious

ble 11. End-Organ Complications of Acute Aortic Dissection

pe End-Organ Complication

rdiovascular Aortic insufficiency

Syncope

Pericardial tamponade

Myocardial ischemia or infarction

Congestive heart failure

urologic Ischemic stroke or transient ischemic attack

Peripheral neuropathy

Paraplegia/paraparesis

Spinal ischemia

lmonary Pleural effusion

Aortopulmonary fistula with hemorrhage

strointestinal Mesenteric ischemia or infarction

Aortoenteric fistula with hemorrhage

nal Renal failure

Renal ischemia or infarction

tremities Limb ischemia

mplication of acute AoD. Registry and review data suggest of

at ECG evidence of myocardial ischemia was present in up19% of patients with acute AoD, whereas pooled data from8 patients in 9 different studies found that acute MI wasesent in 7% of cases (95% CI 4% to 14%) (37,47,250).oronary artery flow can be compromised by an expandinglse lumen compressing the proximal coronary or by exten-on of the dissection flap into the coronary artery ostium51).

Clinically, a dissection-related cardiac malperfusion syn-ome may present with ECG changes that are indistinguish-le from those of primary myocardial ischemia or infarction,creasing the likelihood of misdiagnosis and inappropriateerapeutic intervention (252).

1.5.3. HEART FAILURE AND SHOCK

eart failure is a relatively uncommon complication of AoD,und to occur in approximately 6% of cases (236). In thistting, heart failure may result from acute aortic insuffi-ency, acute myocardial ischemia or infarction, or cardiacmponade. Registry data suggest that patients with acuteoD complicated by heart failure are often atypical in theiresentation, frequently leading to a delay in diagnosis (236).he largest study to evaluate heart failure in acute AoDcluded 1069 patients from the IRAD database and foundat patients with AoD and concomitant heart failure wereore likely to present in shock but were less likely tomplain of chest pain and that, when chest pain was present,e pain was more often mild and less often abrupt in onset36).

1.5.4. PERICARDIAL EFFUSION AND TAMPONADE

ricardial pathology is a frequent complication of acuteype A AoD and can occur via 2 distinct mechanisms7,253–256). Most commonly, transudation of fluid acrosse thin wall of an adjacent false lumen into the pericardialace leads to a hemodynamically insignificant pericardialfusion (256), which is present in about one third of patients57). Less often, the dissected aorta ruptures directly into thericardium, leading rapidly to tamponade physiology andmodynamic compromise (245,258,259). Cardiac tampon-e is diagnosed in 8% to 10% of patients presenting withute Type A AoD and is an ominous clinical predictor ofor outcomes (260), as well as the leading cause of mortalitythis group (47,215,231). Consequently, the presence of

rdiac tamponade should prompt truly urgent aortic repair60).

1.6. Syncopencope is a well-recognized dissection-related complaintcurring in approximately 13% of cases (242,261) withultiple potential etiologies, including: 1) cardiac (e.g.,vere aortic regurgitation, ventricular outflow obstruction,rdiac tamponade), 2) vascular (e.g., impaired cerebralood flow and aortic baroreceptor activation); 3) neurologic.g., vasovagal in response to pain), and 4) volume-related.g., false lumen rupture into the pleural space) causes40,261–267). Regardless of its etiology, syncope in thetting of AoD increases the risk of near-term adverse events.a review of 728 cases of acute AoD, patients with a history
syncope were significantly more likely to die than were

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ose without syncope (34% versus 23%, respectively;�0.01) (242). Additionally, patients who presented withncope more frequently had associated cardiac tamponade,roke, decreased consciousness, and evidence of spinal cordchemia (242).

1.7. Neurologic Complicationscute AoD frequently presents with dissection-related neu-logic complications. Pooled data from more than 1300tients in 13 studies that included both Type A and Bssections found that neurologic symptoms were reported in% (37). Neurologic complications may result from hypo-

nsion, malperfusion, distal thromboembolism, or nervempression (251,253,254). In a recent study of 102 patientsith Type A AoD, 29% had neurologic symptoms on initialesentation (253); of those with neurologic symptoms, 53%d ischemic stroke (predominantly right hemispheric) and% had ischemic neuropathy (described as limb pain withnsory or motor deficit) (253).Last, although uncommon, acute paraplegia as a result ofinal cord malperfusion has been described as a primaryanifestation of thoracic AoD, occurring in 1% to 3% oftients (215,251,253,254,268,269).Of clinical note, up to 50% of dissection-related neurologicmptoms may be transient and as many as one-third oftients with neurologic symptoms present without com-aints of chest pain, complicating appropriate diagnosis andeatment (253,255,256,262,269–271).

1.8. Pulmonary Complicationseural effusion, the most common pulmonary complicationacute AoD, is noted in 16% of cases at presentation (37);

hereas large effusions may result from leaking of bloodom the aorta into the pleural space, small effusions arepically a nonhemorrhagic exudate believed to be inflamma-ry in origin (37,272–275).Other pulmonary complications of acute AoD include

ssection-related compression of the pulmonary artery andvelopment of an aortopulmonary fistula, either of whichay present with dyspnea as a prominent symptom (276–8). Hemoptysis, noted in 1 study in 3% of patientsesenting with thoracic AoD, may result from compressionlung parenchyma by an expanding false lumen or via directeurysmal rupture into the lung, leading to massive hemop-sis and death (215,279,280).

1.9. Gastrointestinal Complicationsesenteric ischemia is the most common gastrointestinalmplication of acute AoD (228) and can result from malp-fusion or systemic hypotension. It is the most commonuse of death among those with Type B AoD. Mesentericchemia is associated with abdominal pain, but pain may benspecific and out of proportion to the physical examinationthe abdomen, so the cause of pain often goes unrecognizedrly on. Unfortunately, by the time serum markers of bowelchemia or infarction turn positive, it is often too late tolvage the bowel or the patient. Therefore, it is essential to

viligant for mesenteric ischemia in every patient with
ute AoD and associated abdominal pain. m
Gastrointestinal hemorrhage is a rare but potentially cata-rophic complication of acute AoD (281–283). Dissection-lated gastrointestinal bleeding may present with limitedeeding as a result of mesenteric infarction or as massivemorrhage secondary to an aortoesophageal fistula or falsemen rupture into proximal small bowel (281–283). Al-ough rare, dissection-related gastrointestinal hemorrhageould be in the differential of all patients presenting witheeding and complaints of thoracic or abdominal pain.

1.10. Blood Pressure and Heart Rate Considerationslood pressure abnormalities are common in patients present-g with acute thoracic AoD. About half of patients arepertensive at presentation, with 71% of Type B patientsving a systolic blood pressure greater than 150 mm Hgrsus only 36% of Type A patients (284–287). Conversely,arly 20% present with either hypotension or shock (37).ypotension and shock can result from cardiac tamponade,rtic hemorrhage, severe aortic insufficiency, myocardial

chemia or infarction, true lumen compression by distendedlse lumen, or an intra-abdominal catastrophe. Of more than00 patients with acute AoD, those with hypotension onmission were found more likely to have neurologic com-ications; myocardial, mesenteric, or limb ischemia; andath (249).Accurate systemic blood pressure measurement may bemplicated by dissection-related occlusion of aortic branchteries, resulting in erroneously low blood pressure readingsthe affected limb. Accordingly, blood pressures may needbe measured in both arms and, at times, both legs to

termine the highest central blood pressure.

1.11. Age and Sex Considerationscute AoD presentation varies with patient age and sex. In1 IRAD patients, 7% were younger than 40 years. Com-red with patients 40 years of age and older, this group wasss likely to have a history of hypertension and significantlyore likely to have Marfan syndrome, bicuspid aortic valve,a history of prior aortic surgery (223). Clinically, young

tients in this study were more likely to describe pain asrupt in onset but less likely to be hypertensive at presen-tion (25% versus 45%) (223). In contrast, a separate studyIRAD data evaluating 550 patients with Type A dissection

und that among patients more than 70 years of age (32% oftal), typical symptoms (abrupt onset of pain) and signsurmur of aortic regurgitation or pulse deficits) were sig-

ficantly less common, suggesting that extra vigilance mayrequired to identify acute AoD in young and elderly

tients (288).Sex appears to affect the presentation of acute AoD as well.a study of 1078 patients enrolled in IRAD, 32% were

omen. Women were older; were less likely to present withinhours of symptom onset, to complain of abrupt onset ofin, and to have a pulse deficit; and were more likely toesent with either altered mental status or congestive heartilure. Consequently, women were less likely to be diag-sed within 24 hours of symptom onset and had significantlygher in-hospital mortality (30% versus 21%, P�0.001) than
en (289).

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.2. Intramural Hematomamong acute aortic syndromes, acute dissection is the mostmmon, but approximately 10% to 20% of patients (290–2) with a clinical picture of dissection exhibit an IMH viaaging without identification of blood flow in a false lumenan intimal lesion. Some believe IMH arises from hemor-

age of the vasa vasorum located within the medial layer ofe aorta (293,294), whereas others argue that the hematomaises from microscopic tears in the aortic intima. Thesulting hematoma may then propagate in an antegrade or atrograde manner, producing symptoms that may be impos-ble to differentiate clinically from those of a classic AoD1). IMH has a variable radiologic appearance (Figure 23)cording to the area of the aorta involved. In some cases,H may be associated with a PAU (see Section 8.2 or 8.3).Clinically, IMH most commonly occurs in the descendingrta and in older patients. Pain is characteristic of IMH,hereas malperfusion and pulse deficit are much less likelyan with classic AoD (295,296).Imaging criteria of IMH are based on the appearance of

esh thrombus in the aortic wall. These include crescentic orrcular thickening of the aortic wall with maximal thicknesseater than or equal to 7 mm on TEE without intimal flap orar or longitudinal flow in the false lumen. The thickenedall has a higher tissue density than unenhanced blood on CTd is without enhancement after contrast on the CT/MR90,296–298). When the term IMH is used strictly, notimal defect such as a tear or an ulcer is present. But inactice, the term is used loosely to mean a thrombosed falsemen regardless of a small intimal defect. The distinction is

gure 23. Intramural hematoma demonstrated as a low-attenuatioft, Axial image at the level of the aortic arch. Top middle, Througtery with narrowing of the aortic lumen. Bottom left, Oblique sagithout the use of ECG gating). Bottom right, Coronal reformattedma, and an incidental infarenal aortic aneurysm. CT indicates co

rther blurred by the facts that the intimal defect may be tiv

btle and difficult to exclude and that some patients withH begin with a CT scan that shows a thrombosed false

men with no apparent intimal defect and then over theurse of 1 or 2 months develop 1 or more distinct ulcerlikemmunications (299,300). Because of this overlap in imag-g findings, it is difficult and perhaps somewhat arbitrary tose treatment on the appearance of the CT snapshot of therta in its disease progression.The natural history of IMH is variable. The hematoma maytirely resolve (10%) (301), it may convert to a classicssection, or the aorta may enlarge and potentially rupture.he clinical behavior of IMH varies according to the locationd mimics that of classic AoD. IMH involving the ascendingrta has a high, early risk of complication and death withedical treatment alone, and surgery is usually indicated.H involving the descending aorta may be treated with

ood pressure control, and the use of beta blockers has beenown to improve the long-term survival rate (296). Conver-on of the IMH to a more classic picture of dissection occurs

3% to 14% of cases involving the descending aorta97,298,302,303) and in 11% to 88% of cases involving thecending aorta (302–305), with that figure increasing withcreased length of follow-up. Progressive increase in aorticameter has been demonstrated by serial imaging studies98,306). In 1 study, the mortality after 2 years of patientsith acute proximal Type A IMH versus that of patients withassic dissection was not significantly different (307). An-her group found improved actuarial survival rates at 1, 2,d 5 years in patients with IMH versus classic dissection:%, 90%, and 90% versus 67%, 66%, and 62%, respec-

d of hematoma (arrows) in the aortic wall on CT images. Topid thorax. Top right, At the level of the superior mesenteric

ormatted image through the thorax (note band artifact evidentthrough the abdomen demonstrate the length of the hema-tomographic imaging; and ECG, electrocardiogram.

n banh the mittal refimage

ely, for Type A (304), and 100%, 97%, and 97% versus

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%, 79%, and 79%, respectively, for Type B (297). Song etalso described increased risk for complications or mortalityr patients with IMH involving the ascending aorta whencending aortic diameter is greater than 4.8 cm or IMHickness is greater than 11 mm (308).

.3. Penetrating Atherosclerotic UlcerU refers to an atherosclerotic lesion with ulceration that

netrates the internal elastic lamina and allows hematomarmation within the media of the aortic wall (309). Thission sets the stage for development of IMH, AoD, or frankssel rupture (309) (Figure 24). Anatomically PAUs developaortic segments where atherosclerotic changes are most

mmon and therefore are localized to the descending tho-cic aorta in over 90% of cases (310). When viewedngentially, the classic appearance of the lesion is aushroom-like outpouching of the aortic lumen with over-nging edges, resembling a gastric ulcer, as depicted on arium study (Figure 24). The typical patient is elderlysually over 65 years of age) and has hypertension andffuse atherosclerosis, who presented with chest or back paint without signs of aortic regurgitation or malperfusion. Lessmmonly, patients presented only with signs of distalbolization (311). Asymptomatic patients may also be

und with aortic lesions that are indistinguishable, by imag-g criteria, from PAUs (310,311).Two entities can mimic PAUs. A branch artery pseudoan-rysm represents a small collection of flowing blood withinotherwise thrombosed aortic false lumen, which is createdan injury to a small branch artery during the propagationthe IMH (312). These are usually incidental findings, are

stinguished from ulcers by the apparent absence of ammunication with the aortic lumen by CT, and do notually require specific treatment. A dissection entry orentry tear may develop in an area of IMH as detected byveral imaging studies over a period of several months97,299,300,306).

.4. Pseudoaneurysms of the Thoracic Aortaeudoaneurysms of the thoracic aorta are frequently relateddeceleration or torsional trauma to the aorta from motorhicle accidents, falls, and sports injuries (3,313–319).ortic pseudoaneurysms are relatively rare, with posttrau-atic pseudoaneurysms having an incidence of 3% to 4%
ter blunt trauma (51). Other pseudoaneurysms may occur ca
llowing aortic surgery, catheter-based interventions, ornetrating trauma. Pseudoaneurysms often have a slimeck” that leads to the “aneurysm” that corresponds toints of penetration and containment (320). Aortic infectionsycotic aneurysms) and penetrating ulcers may also result ineudoaneurysms (see Section 9.2.2). Penetrating injuries areually repaired immediately whenever recognized and fea-ble (317,318,321).

.5. Traumatic Rupture of the Thoracic AortaUK survey of all motor vehicle accident fatalities found

at approximately 20% of patients had an autopsy finding ofruptured aorta, emphasizing the importance of traumaticpture of the aorta (TRA). In the United States, there areound 40 000 motor vehicle deaths annually, and it is likelyat around 8000 of the victims had TRA. It is estimated thatly 9% to 14% of patients with TRA reach a hospital alived only 2% ultimately survive. In this survey, 29% werevolved with frontal impact crashes and 44% were involvedith side impact crashes (318,319).Parmley and colleagues (318) noted the correlation of high

sk of early death and the sites of TRA on the basis oftopsies in 275 deaths from unrelated aortic rupture. In 45%,e tear was at the aortic isthmus; 23%, in the ascendingrta; 13%, in the descending aorta; 8%, in the transverserta; 5%, in the abdominal aorta; and 6%, multiple sites.Examination of the patient usually reveals signs similar toose of coarctation of the aorta with arm blood pressuregher than leg blood pressure, delay between radial versusmoral artery pulsation, and a harsh interscapular murmur). Evidence of polytrauma is, however, common.The best method for detection of a TRA is debated. A chestray with a nasogastric tube in position has 80% sensitivityr suggesting TRA by showing displacement of the naso-stric tube by the hematoma. However, signs of hemome-astinum are more often false positive than true positive0). Even when present, mediastinal blood is less likely to bee to arterial/aortic injury than to less-consequential venouseeding. A biplane contrast aortogram may fail to detect thear until the development of a pseudoaneurysm. TEE may beed, but if dilatation has not occurred, the diagnosis may still

in doubt. CT is used but is not absolutely certain totablish the diagnosis. In questionable cases, intravasculartrasound can also be used (3,322,323) (Figure 8). Realisti-

Figure 24. Penetrating atherosclerotic ulcer of theproximal descending thoracic aorta. Axial CT im-ages at the level of the aortopulmonary window(left) and at the level of the left pulmonary artery(right) demonstrate a small penetrating ulcer (longarrow, U) that extends beyond the expected con-fines of the aortic lumen with adjacent IMH both atthe level of the ulcer itself and that extends a fewcentimeters caudally in the wall of the descendingthoracic aorta (short arrows). CT indicates com-puted tomographic imaging; IMH, intramural he-matoma; and U, penetrating ulcer.

lly, the imaging sequence often depends on the stability of

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e patient and the need for the diagnosis of concomitantjuries. Sometimes, this may even fail to detect the tear, ande study may have to be repeated at a later date to detect thear.

.6. Evaluation and Management of Acuteoracic Aortic Disease

6.1. Initial Evaluation and Management

6.1.1. RECOMMENDATIONS FOR ESTIMATION OF PRETEST

SK OF THORACIC AORTIC DISSECTION

ASS I

Providers should routinely evaluate any patient presenting withcomplaints that may represent acute thoracic aortic dissectionto establish a pretest risk of disease that can then be used toguide diagnostic decisions. This process should include spe-cific questions about medical history, family history, and painfeatures as well as a focused examination to identify findingsthat are associated with aortic dissection, including:a. High-risk conditions and historical features (47,127,223,

261) (Level of Evidence: B):• Marfan syndrome, Loeys-Dietz syndrome, vascular

Ehlers-Danlos syndrome, Turner syndrome, or other con-nective tissue disease.

• Patients with mutations in genes known to predisposeto thoracic aortic aneurysms and dissection, such asFBN1, TGFBR1, TGFBR2, ACTA2, and MYH11.

• Family history of aortic dissection or thoracic aorticaneurysm.

• Known aortic valve disease.• Recent aortic manipulation (surgical or catheter-based).• Known thoracic aortic aneurysm.

b. High-risk chest, back, or abdominal pain features (37,47,215,223,261,264,288) (Level of Evidence: B):• Pain that is abrupt or instantaneous in onset.• Pain that is severe in intensity.• Pain that has a ripping, tearing, stabbing, or sharp

quality.c. High-risk examination features (37,47,253,257,261,324)

(Level of Evidence: B):• Pulse deficit.• Systolic blood pressure limb differential greater than 20

mm Hg.• Focal neurologic deficit.• Murmur of aortic regurgitation (new).

Patients presenting with sudden onset of severe chest, back,and/or abdominal pain, particularly those less than 40 years ofage, should be questioned about a history and examined forphysical features of Marfan syndrome, Loeys-Dietz syndrome,vascular Ehlers-Danlos syndrome, Turner syndrome, or otherconnective tissue disorder associated with thoracic aorticdisease. (223) (Level of Evidence: B)Patients presenting with sudden onset of severe chest, back,and/or abdominal pain should be questioned about a history ofaortic pathology in immediate family members as there is astrong familial component to acute thoracic aortic disease.(223) (Level of Evidence: B)Patients presenting with sudden onset of severe chest, back,
and/or abdominal pain should be questioned about recent se
aortic manipulation (surgical or catheter-based) or a knownhistory of aortic valvular disease, as these factors predisposeto acute aortic dissection. (Level of Evidence: C)In patients with suspected or confirmed aortic dissection whohave experienced a syncopal episode, a focused examinationshould be performed to identify associated neurologic injury orthe presence of pericardial tamponade (see Section 8.1.6).(Level of Evidence: C)All patients presenting with acute neurologic complaintsshould be questioned about the presence of chest, back,and/or abdominal pain and checked for peripheral pulse defi-cits as patients with dissection-related neurologic pathologyare less likely to report thoracic pain than the typical aorticdissection patient (253) (see Section 8.1.7). (Level of Evi-dence: C)

6.1.2. LABORATORY TESTING

veral plasma markers have been investigated for theirility in the evaluation of acute AoD. Plasma smooth muscleyosin heavy chain protein, D-dimer, and high-sensitivity-reactive protein have shown diagnostic promise, although ack of large prospective studies precludes a recommendationgarding their use (325–328).

Elevation of D-dimer levels occurs with intravasculartivation of the coagulation cascade and secondary fibrino-sis and in conditions such as venous thromboembolism,psis, disseminated intravascular coagulation, malignancies,cent trauma or surgery, and acute MI and followingrinolytic therapy. The ACEP has published guidelines

garding the use of certain D-dimer assays to rule outlmonary embolism in low-risk patients (329).

Regarding the potential role of plasma D-dimer levels toreen for AoD, significant elevations of D-dimer were seenall 24 patients with documented acute AoD involving

ther the ascending or descending thoracic aorta regardlesstime from presentation, ranging from 1 to 120 hours (325).meta-analysis of 11 studies (330) noted that the pooled

nsitivity of D-dimer in 349 patients with documented acuteoD was 94% (95% CI 91% to 96%) with specificity rangingom 40% to 100%. Two patients had limited ascending aortic

H without intimal flap and had negative D-dimer assays31).

Some authors (325,332) recommend that D-dimer assaysperformed in all patients where clinical suspicion exists, tolp identify those who do not require definitive imaging

udies. However, the efficacy and safety of this strategy havet been tested in a large clinical trial, and several caveatsould apply. The negative likelihood ratio provided by theost sensitive D-dimer assay is not of sufficient magnitude toovide useful information in high-risk individuals and there-re cannot be used to “rule out” the disease in this group.linical scoring systems to identify the true pretest probabil-

for AoD in individual patients have not been developed orlidated, thus limiting an accurate determination of the trueosttest” probability associated with a negative D-dimersult. Finally, there are reports of negative D-dimer assayssociated with ascending aortic IMH or a thrombosed falsemen, such that further studies are needed regarding the
nsitivity of D-dimer levels to detect the presence of IMH or

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Where a high level of suspicion for acute AoD exists,boratory testing aimed at presurgical screening (bloodunt, serum chemistries, coagulation profiles, and blood typed screen) may reduce preoperative delays.

6.1.3. RECOMMENDATIONS FOR SCREENING TESTS

ASS I

An electrocardiogram should be obtained on all patients whopresent with symptoms that may represent acute thoracicaortic dissection.a. Given the relative infrequency of dissection-related coro-

nary artery occlusion, the presence of ST-segment eleva-tion suggestive of myocardial infarction should be treatedas a primary cardiac event without delay for definitiveaortic imaging unless the patient is at high risk for aorticdissection. (37,47,333) (Level of Evidence: B)

The role of chest x-ray in the evaluation of possible thoracicaortic disease should be directed by the patient’s pretest riskof disease as follows:a. Intermediate risk: Chest x-ray should be performed on all

intermediate-risk patients, as it may establish a clearalternate diagnosis that will obviate the need for definitiveaortic imaging. (Level of Evidence: C)

b. Low risk: Chest x-ray should be performed on all low-riskpatients, as it may either establish an alternative diagnosisor demonstrate findings that are suggestive of thoracicaortic disease, indicating the need for urgent definitiveaortic imaging. (Level of Evidence: C)

Urgent and definitive imaging of the aorta using transesoph-ageal echocardiogram, computed tomographic imaging, ormagnetic resonance imaging is recommended to identify orexclude thoracic aortic dissection in patients at high risk forthe disease by initial screening. (42–46,67,73) (Level ofEvidence: B)

ASS III

A negative chest x-ray should not delay definitive aortic imag-ing in patients determined to be high risk for aortic dissectionby initial screening. (Level of Evidence: C)

6.1.4. RECOMMENDATIONS FOR DIAGNOSTIC

AGING STUDIES

ASS I

Selection of a specific imaging modality to identify or excludeaortic dissection should be based on patient variables andinstitutional capabilities, including immediate availability.(Level of Evidence: C)If a high clinical suspicion exists for acute aortic dissection butinitial aortic imaging is negative, a second imaging studyshould be obtained. (212) (Level of Evidence: C)

6.1.5. RECOMMENDATIONS FOR INITIAL MANAGEMENT

ASS I

Initial management of thoracic aortic dissection should bedirected at decreasing aortic wall stress by controlling heart
rate and blood pressure as follows:
a. In the absence of contraindications, intravenous betablockade should be initiated and titrated to a target heartrate of 60 beats per minute or less. (Level of Evi-dence: C)

b. In patients with clear contraindications to beta blockade,nondihydropyridine calcium channel-blocking agentsshould be used as an alternative for rate control. (Level ofEvidence: C)

c. If systolic blood pressures remain greater than 120 mm Hgafter adequate heart rate control has been obtained, thenangiotensin-converting enzyme inhibitors and/or other va-sodilators should be administered intravenously to furtherreduce blood pressure that maintains adequate end-organperfusion. (Level of Evidence: C)

d. Beta blockers should be used cautiously in the setting ofacute aortic regurgitation because they will block thecompensatory tachycardia. (5) (Level of Evidence: C)

ASS III

Vasodilator therapy should not be initiated prior to rate controlso as to avoid associated reflex tachycardia that may increaseaortic wall stress, leading to propagation or expansion of athoracic aortic dissection. (Level of Evidence: C)

6.1.6. RECOMMENDATIONS FOR DEFINITIVE MANAGEMENT

ASS I

Urgent surgical consultation should be obtained for allpatients diagnosed with thoracic aortic dissection regard-less of the anatomic location (ascending versus descending)as soon as the diagnosis is made or highly suspected. (Levelof Evidence: C)Acute thoracic aortic dissection involving the ascending aortashould be urgently evaluated for emergent surgical repairbecause of the high risk of associated life-threatening compli-cations such as rupture. (47) (Level of Evidence: B)Acute thoracic aortic dissection involving the descendingaorta should be managed medically unless life-threateningcomplications develop (i.e., malperfusion syndrome, progres-sion of dissection, enlarging aneurysm, inability to controlblood pressure or symptoms). (285,288,334–337) (Level ofEvidence: B)

arly identification of acute thoracic dissection is challeng-g. During the initial evaluation, the correct diagnosis ofoD has been made in only 15% to 43% of patients initiallyought to have the disease (215,237,252). Factors that maypede accurate diagnosis of AoD include the following:

Acute AoD is often believed to be a rare disease (2.9 to 3.5cases per 100 000 person-years) (215,217), whereas theincidence of acute MI is several orders of magnitudegreater (more than 200 cases per 100 000 person-years)(338). Data previously cited in this guideline from UHCsuggest that acute AoD is not “rare.” Some commonexplanations that clinicians give for this underlying beliefinclude the following:a. It is difficult for clinicians to effectively separate

patients with AoD from the multitude of other patients
who present to emergency departments and primary

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care physicians with common complaints that aremuch more often not due to acute AoD.

b. Front-line medical providers may have little directexperience with acute AoD and are unlikely to beaware of the subtleties of its presenting signs andsymptoms.

c. Institutional multidisciplinary pathways developed forother emergencies such as ST-elevation myocardialinfarction (STEMI) or acute stroke have not generallyexisted for acute AoD.

In contrast to other more common cardiovascular emer-gencies, acute AoD may occur in younger patients. Accu-rate identification of acute AoD; however, requires thatclinicians recognize on a routine basis that the disease maypresent in younger patients. Case reports exist of childrenas young as 3 years of age presenting with acute AoD(105).Acute AoD may present with a wide range of unusualmanifestations that do not conform with classic “textbookfindings.” (339–341)There are no well-studied, rapidly available, and effectivescreening tests for acute AoD.

.2. Evaluation and Management Algorithmse provided algorithms guide the initial evaluation oftients whose presentations are concerning for AoD and thenagement of patients in whom the diagnosis of acuteracic AoD is confirmed. Although clinicians may ulti-tely choose to deviate from the pathway for patient-cific reasons, the algorithms provide a framework withich to quickly diagnose (Figure 25) and provide earlynagement (Figure 26) of AoD. This decision model isported by several large studies that indicate a targetedtory and physical examination are likely to identify thest majority of patients who present with acute AoD,gesting that adequate screening need not be time intensive

technology dependent (47,215,264). Using a target historyd physical examination, patients can be placed into 1 of 3egories: 1) those with immediately apparent acute AoDuiring emergent surgical evaluation and expedited aorticaging, 2) those whose presentation is concerning for acuteD and in the absence of a clear alternative diagnosisuire expedited aortic imaging, and 3) those whose clinicalsentation is not initially suggestive of acute AoD but may

nefit from aortic imaging in the absence of a likelyernative diagnosis at the completion of the initial evalua-n.Several high-risk conditions (Figure 25, T2–1) greatlyrease the likelihood that presenting complaints that coulda result of acute AoD.Pain (Figure 25, T2–2) is the most commonly reportedsenting symptom of acute AoD regardless of patient age,, or other associated clinical complaint (37,47,215,

3,242,264,289). Pain described as abrupt or instantaneousonset, that is severe in intensity, or that has a ripping orring quality establishes a high pretest probability for AoD,261).

The combination of 2 or more high-risk features (Figure
, T3) is strongly suggestive of acute AoD. acc
The presence of a single high-risk feature (i.e., high-riskndition, pain, or physical examination) may trigger imme-te concern for acute AoD; however, other diagnostic

nsiderations may exist. Pain with high-risk features, al-ugh suggestive of acute AoD, may occur as a result of an

ernate disease process (Table 12). For patients who aretermined to have an intermediate probability of acute AoD

the basis of initial bedside assessment, Figure 25, T4,vides a pathway for further evaluation.

Figure 25, T5, provides a pathway for further evaluation oftients without any high risk features. Delay in diagnosisd increased mortality is common in this group1,288,289).

For patients presenting with new ST-segment elevations oninitial ECG and without high-risk AoD features, imme-

te coronary angiography and reperfusion therapy (i.e.,ombolysis or percutaneous coronary intervention) are in-ated (333). However, if coronary angiography is per-med and no culprit coronary lesion is identified, thenure 25, T6, provides a pathway to dedicated aortic

aging. As approximately 40% of chest films in acute AoDk a widened mediastinum, and as many as 16% are normal,absence of radiographic abnormalities does not excludediagnosis of AoD (37,47) (Figure 25, T7).

Missed or delayed diagnosis of acute AoD is most com-nly ascribed to an incorrect working diagnosis of acute

ronary syndrome, a condition that may require a prolongede interval to correctly identify (i.e., serial cardiac biomark-) and whose management with antiplatlet and antithrombinents may cause harm to the patient with AoD. For patientsth an intermediate-risk profile for acute AoD and who dot have diagnostic STEMI but who are being evaluated forpossible acute coronary syndrome, aortic imaging maytect AoD prior to the administration of antiplatelet andtithrombin agents (Figure 25, T8).Unexplained hypotension is present in approximately 20%patients with acute AoD (37,47). Similarly, a wideneddiastinum on chest x-ray strongly suggests the need for

ditional definitive diagnostic aortic imaging, particularly intients without a clear alternative explanation for theirsenting complaint (342) (Figure 25, T9).Some patients with acute AoD present without any high-k features, making early diagnosis difficult. If a clearernative diagnosis is not established after the initial eval-tion, then obtaining a diagnostic aortic imaging study,rticularly in patients with advanced age (older than 70ars), syncope, focal neurologic deficit, or recent aorticnipulation (surgery or catheter based), should be consid-d (47,242,253,288,343) (Figure 25, T10).Multidetector CT with contrast, TTE, and MR all provideeptable diagnostic accuracy for the diagnosis of acute AoD.patients with hemodynamic instability, requiring close mon-ring, bedside TEE is preferred to avoid moving the patient outthe acute care environment (Figure 25, T11).The most recent comparative study with nonhelical CT, 0.5sla MR and TEE showed 100% sensitivity for all modal-s, with better specificity of CT (100%) than for TEE and

(44). A recent meta-analysis that evaluated the diagnostic
uracy of TEE, helical CT, and MR for suspected AoD

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und that all 3 imaging techniques provided equally reliableagnostic values (46). Accordingly, selection of an imagingodality is influenced by individual patient variables andstitutional capabilities.The diagnosis of acute AoD cannot be excluded defini-ely based on the results of a single imaging study.

lthough TEE, CT, and MR are all highly accurate for thealuation of acute AoD; false-negative studies can and docur (47) (Figures 9 and 15). If a high clinical suspicionists for acute AoD but initial aortic imaging is negative,

rongly consider obtaining a second imaging study (Figure

gure 25. AoD evaluation pathway. ACS indicates acute coronaryrvous system; CT, computed tomographic imaging; CXR, chestEMI, ST-elevation myocardial infarction; TAD; thoracic aortic dis

, T12). er

6.3. Initial Managementnce the diagnosis of AoD or one of its anatomic variantsMH or PAU) is obtained, initial management is directed at

iting propagation of the false lumen by controlling aorticear stress while simultaneously determining which patientsill benefit from surgical or endovascular repair (Figure 26).

6.3.1. BLOOD PRESSURE AND RATE CONTROL THERAPY

ortic wall stress is affected by the velocity of ventricularntraction (dP/dt), the rate of ventricular contraction, andood pressure. Initial medical stabilization using beta block-

ome; AoD, aortic dissection; BP, blood pressure; CNS, centralEKG, electrocardiogram; MR, magnetic resonance imaging;nd TEE, transesophageal echocardiogram.

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ood pressure to the lowest amounts that will still maintainequate end-organ perfusion (61). Reasonable initial targetse a heart rate less than 60 bpm and a systolic blood pressuretween 100 and 120 mm Hg (61).

Intravenous propranolol, metoprolol, labetalol, or esmololan excellent choice for initial treatment. In patients whove a potential contraindication to beta blockade (e.g., those

gure 26. Acute AoD management pathway. AoD indicates aorticE, transthoracic echocardiogram.

ith asthma, congestive heart failure, or chronic obstructive (v

lmonary disease), esmolol may be a viable option given itstremely short half-life. Use of labetalol, which is both anpha- and beta-receptor antagonist, offers the advantage oftent heart rate and blood pressure control from a singleent, potentially eliminating the need for a secondarysodilator. In patients who are unable to tolerate betaockade, nondihydropyridine calcium channel antagonists

tion; BP, blood pressure; MAP, mean arterial pressure; and
dissec
erapamil, diltiazem) offer an acceptable, although less-

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tablished, alternative (61). Beta blockers, verapamil, orltiazem for rate control in patients with significant aorticgurgitation may be problematic because of deleteriousfects on reflex tachycardia.

6.3.2. ADDITIONAL ANTIHYPERTENSIVE THERAPY

is frequently difficult to reduce blood pressure to optimumvels (285–287,344–346). In 1 study, patients required aedian of 4 different antihypertensive drugs (347). In addi-n to beta blockade, vasodilators may be required to control

ood pressure. Intravenous sodium nitroprusside is the mosttablished agent and offers the advantage of being rapidlyratable (61). Nicardipine (348), nitroglycerin, fenoldopam,d various other intravenous antihypertensive agents arepropriate for this situation. Vasodilator therapy withoutior beta blockade may cause reflex tachycardia and in-eased force of ventricular contraction leading to greaterrtic wall stress and potentially causing false lumen propa-tion (42). Following initial stabilization with intravenoustihypertensives, most patients will require long-term anti-pertensive treatment including the use of a beta blockerus additional classes of agents. Angiotensin-convertingzyme inhibitors or angiotension receptor blockers maytard aortic dilatation and their use may be indicated astlined in Section 9.2.1.1.

6.3.3. PAIN CONTROL

dequate pain control is essential in the setting of acute AoDdecrease sympathetic mediated increases in heart rate and

ood pressure. Appropriate use of intravenous opiate anal-sia will help augment the effects of rate control andsodilator agents.

6.3.4. HYPOTENSION

edical management options for all forms of dissection-

ble 12. Differential Diagnosis for High-Risk Pain oramination Features

est pain

● Acute myocardial infarction

● Pulmonary embolism

● Spontaneous pneumothorax

● Esophageal rupture

dominal pain

● Renal/biliary colic

● Bowel obstruction/perforation

● Non–dissection-related mesenteric ischemia

ck pain

● Renal colic

● Musculoskeletal pain

● Intervertebral disk herniation

lse deficit

● Non–dissection-related embolic phenomena

● Non–dissection-related arterial occlusion

cal neurologic deficit

● Primary ischemic cerebrovascular accident

● Cauda equina syndrome

lated hypotension are limited. Volume administration ti- us

ated to improvement of blood pressure is a reasonable firstproach. Vasopressors can be added, if needed, to maintainequate perfusion but have the potential to cause furtherlse lumen propagation. Inotropic agents are likely to in-ease the force and rate of ventricular contraction anderefore increase sheer stress on the aortic wall.

Pericardiocentesis for dissection-related hemopericardiums been associated with recurrent pericardial bleeding andsociated mortality (307,349,350). Several articles from thesian literature suggest that pericardiocentesis may be safe ine setting of acute Type A IMH (351,352). Other cardiacmplications that may result in hypotension include severessection-related aortic regurgitation, true lumen obstruction

a compressing false lumen, and acute MI. All requirefinitive operative management. Hypotension or shock ine setting of AoD may also result from contained rupture ofe false lumen into adjacent structures (i.e., pleural space orediastinum), a scenario that also mandates immediate op-ative intervention.

Ultimately, hypotension or shock in the acute AoD patientggests the need for immediate operative management. Fortients with hemopericardium and cardiac tamponade whonnot survive until surgery, pericardiocentesis can be per-rmed by withdrawing just enough fluid to restore perfusion.

6.3.5. DETERMINING DEFINITIVE MANAGEMENT

the clinically stable patient, the decision for surgical versusedical management of patients with acute AoD is basedimarily on the location of the dissection as described by theanford and DeBakey classification systems (61,353) (seection 8.1.2). A prompt cardiac surgical consultation pro-des the best management resource, regardless of location ofe AoD, as it is impossible to predict which complicationsay develop or when they may occur.

6.4. Recommendation for Surgical Intervention forcute Thoracic Aortic DissectionASS I

For patients with ascending thoracic aortic dissection, all ofthe aneurysmal aorta and the proximal extent of the dissec-tion should be resected. A partially dissected aortic rootmay be repaired with aortic valve resuspension. Extensivedissection of the aortic root should be treated with aorticroot replacement with a composite graft or with a valvesparing root replacement. If a DeBakey Type II dissection ispresent, the entire dissected aorta should be replaced.(Level of Evidence: C)

hen a Type A AoD involves the aortic root, resuspension ofe valve with preservation of the aortic sinuses and excision

the sinuses and resuspension of the valve within alyester graft are suitable options. If the aortic root islated, or if there is extensive dissection and disruption of thertic sinuses, replacement with a composite graft is neces-ry.

6.5. Endovascular Interventionsndovascular stent grafts are not approved for AoD involvinge ascending aorta or aortic arch. Endovascular stent grafts
ed for descending thoracic aortic dissection is discussed in

Secu

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ction 9.2.2.3.1. Indications for either surgical or endovas-lar interventions are discussed in Section 9.

6.6. Principles of Treatment for Intramuralematoma and Penetrating Atherosclerotic Ulcer

he goals of treatment are to prevent aortic rupture orogression to classic AoD, allow patient stabilization beforegent surgery, and reduce complexity of unavoidable aorticrgery. Aggressive medical treatment usually includes, par-ularly in symptomatic patients, beta blockers and othertihypertensive therapy. Indications for open or endograft

eatment are based on the anatomic features of the lesion,inical presentation and course, patient comorbidities, andatomic constraints related to endograft technology. Treat-ent by endografts or open aortic reconstruction can bescussed in the context of 3 overlapping aortic lesions:timal defect without IMH, intimal defect with IMH, andH without an intimal defect.

6.6.1. INTIMAL DEFECT WITHOUT INTRAMURAL HEMATOMA

hese are localized lesions and may involve a limitedgment of the aorta. They are often an incidental finding. Byaging criteria, they include uncomplicated aortic ulcers,

ebs, and eccentric or saccular aneurysms of the aorta. Theye treated as saccular aneurysms based on their maximumameter and clinical feature (212,306,354). These lesionsn be treated with open reconstruction and are the mostitable of the 3 groups for treatment by endografts, if in thescending thoracic aorta. They involve a limited segment,hich can easily be excluded from the circulation, as long asere is an adequate distance from a critical branch artery.hen these limited dissections involve the ascending aorta,ergency surgery is indicated as for other types of AoD

ecause rupture or cardiac tamponade can occur12,306,354).

6.6.2. INTIMAL DEFECT WITH INTRAMURAL HEMATOMA

he intimal defect again presents a target lesion for endovas-lar treatment in the descending thoracic aorta, but thesociated IMH involves a longer segment of aorta than thest category. If the patient becomes asymptomatic in re-onse to aggressive medical treatment, it may be possible tolay endovascular or open reconstruction until the IMH hasabsorbed and organized. (Of note, some writing committeeembers have observed healing of IMH such that immediateconstruction was not required but have continued to followat small number of patients closely.) Two considerationsfect the length of aorta bordering the intimal defect, which

to be included in the segment targeted for treatment.vidence of adjacent atheromatous wall should favor moretensive treatment of the aorta with longer endografts,cause radiographic imaging underestimates shallow ulcer-ed atheromas, and the ulcer typically arises in a bed ofheromatous intima. Treatment with longer endografts pro-des a safety margin against undertreating the intimal defect.he second consideration is the extent of associated IMH.he self-expanding endograft may tear through the intimal
rface into underlying thrombosed false lumen. When treat- sa
ent of this lesion in the acute stage is clinically necessary.g., persisting pain, evidence for expansion or rupture,mpromise of critical branches), it is preferable to anchore endograft in the noninvolved wall above and below thetimal defect.

6.6.3. RECOMMENDATION FOR INTRAMURAL HEMATOMA

ITHOUT INTIMAL DEFECT

ASS IIa

It is reasonable to treat intramural hematoma similar to aorticdissection in the corresponding segment of the aorta. (Level ofEvidence: C)

s noted earlier, some authors suggest treating IMH as anoD in the corresponding aortic territory. Others recommendvasive treatment regardless of location or aortic diameter54). However, small patient series, incomplete anatomicscription of case material, and lack of explicit anatomic orinical guidelines indicating open or endovascular aorticpair make it difficult to generalize from the literature.

The absence of an intimal defect, which can serve as arget lesion, presents a diagnostic as well as treatmentallenge. Intimal tears can be extremely subtle, dependingthe size of the intimal tear and the amount of intramedial

rombus, which can sometimes fill the cavity flush with thertic lumen. The intimal tear may be remote in the aortaspite leaking into the chest. IMH in a normal caliber aortaithout an apparent intimal tear precludes limited treatmenta target lesion. There are no data supporting prophylacticplantation of endografts covering the entire descendingrta, yet in unusual circumstances one may be forced toopose such treatment. IMH in an aneurysmal aorta presentsparticularly urgent problem, because this complication may

a precursor to aneurysm rupture. Although the literatureves no compelling guidelines for treatment, the writingmmittee believes that treatment of IMH corresponding to

eatment of AoD in the corresponding segment of the aortareasonable.

.7. Treatment for the Management ofaumatic Aortic Rupture

he management of blunt TRA is evolving (3,318,319,5,356). On the basis of the report by Parmley and col-

agues (318), most surgeons have recommended immediatergical repair. However, when other serious traumatic inju-

es are present including head injuries and long bone orlvic fractures, immediate surgery may not be feasible oray be dangerous. Multiple studies appear to show that ifreful blood pressure control is used, many patients can be

eated initially conservatively and then undergo operationce their other injuries have been stabilized (3,321,357–9). In a review by Svensson et al (359) of 44 patientsitially treated with careful blood pressure control whobsequently had delayed open surgery, there were no oper-ive deaths. This approach has also been reported as being
fe by Pate and colleagues and others (3,321,357,358). Thus,

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selected patients at high risk for other injuries andeeding, delayed repair of traumatic contained rupture of therta may be an option.The open surgical repair of TRA has evolved over time.a meta-analysis of 596 TRA patients by Svensson et al

59), the highest mortality rate was noted with cardiopul-onary bypass (16.7%), the rate was less with shunts1.4%), and the rate was lowest with a simple “clamp andw” approach (5.8%, P�0.01). There was no difference ine risk of postoperative paralysis. Subsequently, vonppell and colleagues (360) reviewed 1742 patients andund the risk of death was 18.2% with cardiopulmonarypass, 11.9% for distal perfusion with atriofemoral by-ss, 12.3% for shunts, and 16% for the “clamp and sew”ethod. The respective paralysis rates were 2.4%, 1.7%,.1%, and 19.2%, respectively. The key factor in openpairs has been to keep the total aorta cross-clamp time to

short a period as possible, especially less than 45inutes (3,359,361–364).The latest evolution in managing TRA is the use ofdovascular deployed graft covered stents. Althoughdovascular stent grafting has not been prospectively

udied for this clinical scenario, US Food and Drugdministration–approved devices are being used “off la-l,” with considerable success reported based on retro-ective studies. In a collected series of 284 patientsported in the literature, Lettinga-van de Poll and col-agues reported the procedure-related mortality was 1.5%,7% had endoleaks, and 14.4% had procedure-relatedmplications (358,365–370). In a multicenter study of 30tients with 100% implantation success, 6% to 7% oftients died, 1 had a stroke (3.3%), and 1 had partial stentllapse (3.3%) (370). Reporting bias of favorable resultsay be an issue regarding interpretation of the safety andficacy of this approach.The problems with endovascular grafting for TRA havecluded the need to cover the left subclavian artery; the acutearp angle of the distal aortic arch, particularly in youngtients; and the lack of sufficiently small prostheses for useyoung patients. The size and angle problem can result in

e “bird beak” deformity, where the proximal edge of theent is not in contact with the aortic wall and can result inting or collapse of the stent (366) (Figure 27). Similarly,hen the stent graft is larger than the aortic diameter,folding of the stent and collapse can occur. On cross-ctional views, the stent graft has the appearance of aagrammatic heart.Over time, it is hoped newer iterations of endografts will

developed that are better able to accommodate thegulation of the distal arch and are smaller. It is unlikelyat a prospective randomized study will be performed foris because of the small number of patients who make it

any surgical center and because initial results withdografting of TRA, with exception of the problems

sted earlier, have been reasonable. The Expert Opinionommittee of the Society of Thoracic Surgeons and themerican Association of Thoracic Surgeons suggested thatth acute and chronic ruptures be considered for treat-
ent with endografts. Some measures of caution must be al
ken because these are young patients who may bebjected to cumulative radiation exposure with multipleT scans and because the long-term durability of endo-scular stent grafts is not known (371).

. Thoracic Aortic Aneurysms

ost thoracic aortic aneurysms are caused by degenerativesease resulting in dilatation of the aorta (Figure 28). Thecidence of thoracic aortic aneurysms is estimated to becreasing and there are around 10.4 cases per 100 000rson-years (372).Risk factors for development of thoracic aortic aneurysmsclude hypertension, smoking, and chronic obstructive pul-onary disease. In addition, several genetic syndromes withpredisposition for thoracic aortic aneurysms have beenentified and are listed in Section 5. Thoracic aortic aneu-sms are also associated with bicuspid aortic valve (seection 6.1) and other congenital cardiovascular anomalies

ee Section 6) and inflammatory diseases (see Section 7).me thoracic aortic aneurysms are due to an inheritance ofpredisposition for the disease, termed familial thoracicrtic aneurysm syndrome (see Section 5.1.6), and still otherse idiopathic.Many patients with a thoracic aortic aneurysm are asymp-matic and diagnosed by chest x-ray or CT scan obtained forher reasons. An aneurysm may cause compressive symp-ms on adjacent structures including hoarseness, from leftcurrent laryngeal nerve stretching; stridor, from tracheal oronchial compression; dyspnea, from lung compression;sphagia, from esophageal compression; and plethora andema, from superior vena cava compression. Aortic valvegurgitation may develop due to aortic root or ascendingrtic dilatation and result in heart failure. Neck and jaw painay occur with aortic arch aneurysms, whereas back, inter-apular, and/or left shoulder pain may occur with descendingoracic aortic aneurysms. Embolization of atheroscleroticbris with end-organ symptoms may occur. Finally, acutendromes including dissection or rupture without dissectionay occur with potentially catastrophic outcomes as de-ribed in Section 8.5.Thoracic aortic aneurysms may involve different seg-ents of the aorta. The ascending thoracic aorta and/orot is most commonly involved, with the descending aortavolved less often. Involvement of the aortic arch occursonly 10%. The etiology, natural history, and treatments

ffer somewhat for aneurysms in each location. In Marfanndrome, aneurysms typically arise in the aortic root, aocess often referred to as annuloaortic ectasia. Becausee leaflets of the aortic valve are suspended within theot, successful repair of the aortic root may requirerformance of a valve-sparing root repair or, in someses, a composite aortic graft.The average rate of expansion of thoracic aortic aneurysmsestimated to be 0.10 to 0.42 cm/y (373–375). Medical andrgical treatment considerations and selection criteria areted in Section 9.2.A leaking or ruptured aneurysm (see Section 9.1.2.1) may

so present as chest pain with hypotension due to hemor-

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age into the left or right pleural space or pericardium (376);aortoesophageal fistula may manifest as gastrointestinal

morrhage (377). An unusual manifestation reported is amoptysis from a ruptured ascending aortic aneurysm erod-g into the left lung bronchus (378).Further anatomic classifications refer to segments of thescending thoracic aorta and thoracoabdominal aortavided into subsections according to the extent of thesease that is replaced at the time of surgery. Thesetents have an important influence on the risk of deathd complications after surgery or stenting (Figure 29)

gure 27. Beaking of thoracic endoprosthesis. Top left, Baselineatic pseudoaneurysm several cm distal to the left subclavian arte

mm, and distal to the pseudoaneurysm measured 21 mm. Topeter cuff distally and a 26 mm � 10 cm thoracic endograft prox

esent (arrow), with lack of conformity of the proximal endograft was covered intentionally to exclude the pseudoaneurysm, which dwing implantation of the endografts shows collapse of the leadinntimeters more caudally, the endograft remains collapsed postew). Bottom middle, Thoracic aortography confirms posterior collaht, After placement of a self-expanding z-stent in the proximal een reexpanded and the pseudoaneurysm was once again excludnded and the pseudoaneurysm has remained excluded for 3 yea

ee Section 9.2.2.3).

.1. General Approach to the Patient

1.1. Recommendation for History and Physicalxamination for Thoracic Aortic DiseaseASS I

For patients presenting with a history of acute cardiac andnoncardiac symptoms associated with a significant likelihoodof thoracic aortic disease, the clinician should perform afocused physical examination, including a careful and com-plete search for arterial perfusion differentials in both upperand lower extremities, evidence of visceral ischemia, focal

c aortography in left anterior oblique projection shows a trau-rtic diameter proximal to the left subclavian artery measured, Thoracic aortography following deployment of a 23-mm-di-Considerable beaking of the leading edge of the endograft istight inner curve of the aortic arch. The left subclavian artery

ot opacify. Top right, CT examination of the chest 2 days fol-of the endograft (arrow). Bottom left, On a CT slice severallly, resulting in revascularization of the pseudoaneurysm (ar-

f the endograft and reopening of the pseudoaneurysm. Bottomft and reballooning of the endoprostheses, the endograft hasthough beaking persists, the endograft has remained fully ex-ollow-up. CT indicates computed tomographic imaging.

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and findings compatible with possible cardiac tamponade.(339–341) (Level of Evidence: C)

he physical findings of thoracic aortic diseases may bebtle indirect manifestations of uncommon underlying ge-tically predisposing conditions. In evaluating the evidencese for physical examination of patients with thoracic aorticsease, there are no controlled or blinded experimentalsearch studies that have stratified patients into differenteatment categories based on physical findings.For all patients with thoracic aortic disease, the first andremost issue is to identify those who are acutely at risk for
tastrophic harm as early as possible. Establishing a set of et
riggers” or “red flags” may serve as alerts to either excludeidentify life-threatening thoracic aortic disease.Given the growing awareness of an extensive variety of

seases associated with nonemergent thoracic aortic disease,is important to be aware of the many different physicaldings associated with extracardiovascular etiologies partic-

arly those of genetic origin (see Section 5).

1.1.1. CORONARY ARTERY DISEASE

he frequency of coexisting CAD varies widely amongtient subgroups with thoracic aortic disease as does the

Figure 28. Ascending thoracic aortic aneurysm ina patient with calcific aortic stenosis. Top left, Ax-ial CT image demonstrates an enlarged ascendingthoracic aorta (A) and normal caliber descendingthoracic aorta (D). Top right, Axial CT image dem-onstrates extensive aortic valve leaflet calcification(arrows). Middle left, Coronal CT image also dem-onstrates the dilated ascending aorta (A) and aor-tic valve leaflet calcification (arrows). Middle right,Volume rendered CT image demonstrates the di-lated ascending thoracic aorta (A), normal caliberaortic arch and descending thoracic aorta (D) andgreat vessels with a bovine arch configuration(INN, LCCA, LSCA). Bottom, Volume rendered ro-tating image of the thoracic aorta can be used todepict the anatomy, particularly the relationship ofan aortic abnormality to the great vessels, for sur-gical planning. See the accompanying full cinevideo for the bottom panel. CT indicates com-puted tomographic imaging; INN, innominate ar-tery; LCCA, left common carotid artery; LSCA, leftsubclavian artery; and LV, left ventricle.

iology of the coronary artery abnormalities, if present.


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tients with atherosclerotic aneurysms of the descendingrta are at elevated risk for coronary atherosclerosis, partic-arly if they have multiple atherosclerotic risk factors. Whiletients with Type A dissection or annuloaortic ectasia mayprotected from atherosclerosis (383), patients with Taka-

su arteritis may occasionally have inflammatory coronaryvolvement with coronary aneurysms (less than 10%)84,385). Similarly, an occasional patient with GCA mayve coronary artery involvement (386,387). If ascendingrtic surgery is being considered, with or without aorticlve surgery, then identification of the coronary anatomyd any underlying CAD is important for planning the besteration.

1.1.2. EMBOLI

mbolization of material thrombus, atheromatous debris, orolesterol crystals may affect any distal arterial bed (seection 11.3). Embolization may occur in patients withoracic aortic aneurysms or atheromas and in those whove undergone angiography, major vessel surgery, or throm-lytic therapy (388–397). Clinical consequences of suchbolization vary considerably, from being completely

ymptomatic to presenting with acute multiorgan failure,cluding progressive renal failure or cutaneous involvement,ith a mortality rate as high as 70% to 90% (398).

1.1.3. ASSOCIATED RENAL ISCHEMIA

enal complications of thoracic aortic disease may be acute,bacute, and chronic (399). Patients may present with severedominal or flank pain, hematuria, fever, nausea, or ambination of these signs and symptoms (400).

1.1.4. ASSOCIATED MESENTERIC ISCHEMIA

tients with acute intestinal ischemia have severe abdominal
in that is initially out of proportion to physical findings to
01). Hours to days later, peritonits and sepsis correlate withtestinal perforation. Findings suggestive of intestinal isch-ia as well as specific arterial or venous obstruction require

rther surgical or vascular specialist evaluation (402–406).

1.1.5. ASSOCIATED PERIPHERAL ISCHEMIA

cute limb ischemia results in pain, pallor, paraesthesias, andralysis (4). Noninvasive vascular diagnostic testing (e.g.,kle- and toe-brachial indices, segmental pressure measure-ents, pulse volume recordings, duplex ultrasound imaging,d Doppler waveform analysis) may document ischemiaith additional use of angiographic imaging when necessary07).

1.2. Differential Diagnosis

1.2.1. SYMPTOMS

mptoms are most commonly related to pain or discomfort.rticularly large thoracic aneurysms may be associated withest discomfort. Rarely, dysphagia (dysphagia lusoria) orspnea is present, usually related to congenital distal arch

sions, such as aberrant right subclavian artery and Kom-erell diverticulum or Felson and Palayew Type I or IIght-sided aortic arch lesions (408).

History of fevers may be related to inflammatory disease orycotic aneurysms. Occasionally, with chronic dissectiond leaking aneurysms, the reabsorption of blood may besociated with fever or jaundice.

1.2.2. PHYSICAL FINDINGS

ost physical findings are not specific for thoracic aorticsease. Other findings may be related to genetic syndromesd connective tissue disorders (see Section 5) or inflamma-

Figure 29. Descending aneurysm classification.Descending aneurysms are classified as involvingthirds of the descending thoracic aorta and variouscombinations. A involves the proximal third, B themiddle third, and C as the distal third. Thus, ananeurysm involving the proximal two thirds is anAB extent aneurysm. Practically, these groupingscan be combined into proximal or distal aneurysm,because these extents influence the risk of paraly-sis after either open or endovascular repairs. Tho-racoabdominal aneurysms are classified accordingto the Crawford classification: Type I extends fromproximal to the sixth rib and extends down to therenal arteries. Type II extends from proximal to thesixth rib and extends to below the renal arteries.Type III extends from distal to the sixth rib butfrom above the diaphragm into the abdominalaorta. Type IV extends from below the diaphragmand involves the entire visceral aortic segment andmost of the abdominal aorta. Juxtarenal and su-praenal aneurysms are excluded (379–382). Imagereprinted with permission from the Cleveland ClinicFoundation.

ry diseases (see Section 7). Findings associated with coarc-

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1.3. Considerations for Imagingecause most cases of chronic thoracic aortic disease areymptomatic and difficult to detect on physical examination,e clinician must have a low threshold for screening fororacic aortic disease. CT or MR is required to adequatelysualize the affected aorta. There has been no cost–benefitalysis of screening in these populations (see Section 18.1).

.2. General Medical Treatment and Riskactor Management for Patients Withoracic Aortic Disease

2.1. Recommendation for Medical Treatment ofatients With Thoracic Aortic DiseasesASS I

Stringent control of hypertension, lipid profile optimization,smoking cessation, and other atherosclerosis risk-reduction

ble 13. Studies of Medical Treatment of Thoracic Aortic Aneur

eatment Studies

ta blockers Genoni M, Paul M, Jenni R,et al (410)

Retrospectivmedical treaother antihy20 (45%) frincidence o40% (8 of 2

Shores J, Berger KR, Murphy EA,et al (88)

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Ahimastos AA, Aggarwal A,D’Orsa KM, et al (412)

Randomizedtaking beta-perindopril-the ascendireduction inrespectively

giotensin receptorckers

Mochizuki S, Dahlof B, Shimizu M,et al (413)

3081 Japancombinationother treatmreduction inin aortic dis

Brooke BS, Habashi JP, Judge DP,et al (89)

The clinicalin 1 patientroot enlargesignificantlyduring angioenlargemenmean differangiotensionchange in ddistal ascen

atins Diehm N, Decker G, Katzen B,et al (414)

A nonrandoof patientsStatin use wHR 0.613, 91.795, 95%

AAA indicates abdominal aortic aneurysm; CI, confidence interval; SD, standa

measures should be instituted for patients with small aneu- po

rysms not requiring surgery, as well as for patients who are notconsidered to be surgical or stent graft candidates. (Level ofEvidence: C)

he patient’s general status should be optimized wheressible. Respiratory illness, a common comorbid problem,ay be improved by stopping smoking, clearing bronchitis,d exercising regularly with walking. Because other athero-lerotic disease is usually present in patients with thoracicrtic aneurysms or atheroma, risk-reduction measures astlined in other guidelines are appropriate (409). Additionaledical management rationales are noted in Table 13.Patients who are not candidates for operative intervention

clude those whose aneurysms or other aortic pathology dot meet the criteria for surgical intervention and those in

hom the criteria are met but who are considered inoperable,ost commonly because of coexisting disease. Patients withrge aneurysms who are considered inoperable may benefitom stringent control of risk factors (see Section 3.2) to

Results

-record review of 78 patients with chronic Type B dissection who received51 of 71 received beta-blocker treatment, 20 of 71 were treated withve drugs. 10 of 51 (20%) of the beta-blocker–treated patients and 9 ofother treatment group needed dissection-related surgery (P�0.002). Theing aortic diameter was 12% (6 of 51) in the beta-blocker group ande other treatment group (P�0.002).

ized, control study of propranolol in 70 patients with Marfan syndrome.eceived a mean daily propranolol dose of 212�68 mg/d. Propranololic root dilation (0.023 vs 0.084 per year, P�0.001).

ation of aortic dilation in children with Marfan syndrome. Aortic dilatationmm/y in children treated with beta blockers.

-blind, placebo-controlled trial of 17 patients with Marfan syndrometherapy to perindopril or placebo. After 24 weeks of therapy, theubjects compared with placebo-treated subjects had smaller growth indiameter during systole (1.2 vs 0.3 mm/m2, P�0.01) and a significant

ing aortic diameter during diastole (0.4 vs �1.2 mm/m2, P�0.001),

ients with hypertension, coronary heart disease, heart failure, or aandomly assigned either to open-label valsartan (40 to 160 mg/d) or tohout angiotension receptor blockers. Patients randomized to valsartan hadsite cardiovascular outcome (OR 0.61, 95% CI 0.47 to 0.79) and reduction(OR 0.18, 95% CI 0.04 to 0.88). Open-label, randomized.

e to angiotension receptor blockers (losartan in 17 patients and irbesartanvaluated in pediatric patients with Marfan syndrome with severe aortiche mean (�SD) rate of change in aortic root diameter decreased.54�2.87 mm/y during previous medical therapy to 0.46�0.62 mm/yreceptor blocker therapy (P�0.001). The deviation of aortic rootormal, as expressed by the rate of change in z scores, was reduced by a1.47 z scores/y (95% CI 0.70 to 2.24, P�0.001) after the initiation ofor blocker therapy. The sinotubular junction showed a reduced rate ofduring angiotension receptor blocker therapy (P�0.05), whereas the

rta was not affected by angiotension receptor blocker therapy.

ropensity-score–adjusted study of statin use effect on long-term mortalityovascular repair of AAA (731 patients) or TAA (59 patients) was done.ciated with decreased long-term mortality in patients with AAA (adjusted.379 to 0.993, P�0.047), but not for patients with TAA (adjusted HR7 to 21.942; P�0.647).

ation; and TAA, thoracic aortic aneurysm.

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pture or dissection. Recommendations for periodic imaginge noted in Section 14.

2.1.1. RECOMMENDATIONS FOR BLOOD

ESSURE CONTROL

ASS I

Antihypertensive therapy should be administered to hyperten-sive patients with thoracic aortic diseases to achieve a goal ofless than 140/90 mm Hg (patients without diabetes) or lessthan 130/80 mm Hg (patients with diabetes or chronic renaldisease) to reduce the risk of stroke, myocardial infarction,heart failure, and cardiovascular death. (415–419) (Level ofEvidence: B)Beta adrenergic–blocking drugs should be administered to allpatients with Marfan syndrome and aortic aneurysm to reducethe rate of aortic dilatation unless contraindicated. (88) (Levelof Evidence: B)

ASS IIa

For patients with thoracic aortic aneurysm, it is reasonable toreduce blood pressure with beta blockers and angiotensin-converting enzyme inhibitors (412) or angiotensin receptorblockers (89,413) to the lowest point patients can toleratewithout adverse effects. (88,410,411) (Level of Evidence: B)An angiotensin receptor blocker (losartan) is reasonable forpatients with Marfan syndrome, to reduce the rate of aorticdilatation unless contraindicated. (89,90) (Level of Evi-dence: B)

reatment of hypertension to reduce end points of MI, stroke,d death is well established with many randomized clinical

ials (420). In the Jikei Heart Study, Japanese patients whoceived valsartan along with other antihypertensive therapyd a significantly lower rate of cardiovascular morbidity andortality compared with patients treated without valsartan.eductions noted in particular included lower incidence ofroke, transient ischemic attack (TIA), angina pectoris, andart failure. Moreover, pertinent to this guideline, there wassignificant reduction in the incidence of AoD in thelsartan-treated patients, which contributed to the reductionoverall cardiovascular morbidity and mortality (413).

Currently, beta adrenergic blockade serves as the founda-n of the medical regimen because of demonstrated inhibi-n of aneurysm expansion in patients with Marfan syn-ome. Shores and colleagues (88) randomized 70 patientsith Marfan syndrome to propranolol or placebo in a open-bel study demonstrating an attenuated rate of expansioner the 10-year follow-up. Dietz and colleagues (91) dem-strated that angiotensin receptor blocker therapy reduceseurysm expansion in animal models of Marfan syndrome.

his group has also recently demonstrated that angiotensionceptor blocker therapy slowed the rate of progression ofogressive aortic root dilatation in a preliminary study of 18diatric patients with Marfan syndrome (89). Both betaockade and angiotensin II receptor blockade therapy areing further investigated in a randomized trial for patientsith Marfan syndrome (90).

Lifestyle modifications of diet, weight reduction for over-eight or obese patients, moderation of alcohol consumption,
d aerobic exercise are standard approaches to treat hyper- pl
nsion (421), but pharmacological therapy is usually re-ired for patients with thoracic aortic diseases.

2.1.2. RECOMMENDATION FOR DYSLIPIDEMIA

ASS IIa

Treatment with a statin to achieve a target LDL cholesterol ofless than 70 mg/dL is reasonable for patients with a coronaryheart disease risk equivalent such as noncoronary atheroscle-rotic disease, atherosclerotic aortic aneurysm, and coexistentcoronary heart disease at high risk for coronary ischemicevents. (422–425) (Level of Evidence: A)

he National Cholesterol Education Program ATP III recom-ends that patients with noncoronary atherosclerosis beeated like patients with established coronary heart disease26). Atherosclerosis in the aorta, like atherosclerosis in anyncoronary vascular bed, markedly increases the risk of MId stroke. As a result of this high-risk status (greater than% event rate in 10 years), the goal for hypolipidemicerapy is an LDL level less than 100 mg/dL. Initial therapy

these patients should be a statin. After the Nationalholesterol Education Program ATP III guidelines wereleased in 2001, the Heart Protection Study reported in 2002at patients with atherosclerosis and a total cholesterol leveleater than 135 mg/dL benefited from the addition ofmvastatin 40 mg/d (427). The RR reductions remained evenhen LDL started at less than 100 mg/dL. In concert withta from patients with acute coronary syndromes, the morecent ACC/AHA Guidelines for the Management of Patientsith Peripheral Arterial Disease also gave a Class IIacommendation suggesting the use of a statin to achieve arget LDL of less than 70 mg/dL for patients at very high riskischemic events is reasonable (4).

There are experimental data demonstrating a delayedvelopment of atherosclerosis and prevention of aneurysmvelopment by statins (428–430). However, there are noinical outcomes data that justify their use acutely or suggestat statins prevent expansion after thoracic aortic aneurysmsve developed.

2.1.3. RECOMMENDATION FOR SMOKING CESSATION

ASS I

Smoking cessation and avoidance of exposure to environmen-tal tobacco smoke at work and home are recommended.Follow-up, referral to special programs, and/or pharmacother-apy (including nicotine replacement, buproprion, or vareni-cline) is useful, as is adopting a stepwise strategy aimed atsmoking cessation (the 5 A’s are Ask, Advise, Assess, Assist,and Arrange). (431–432b) (Level of Evidence: B)

here are no randomized or prospective trials that havevestigated the effect of smoking cessation on thoracic aorticsease. Patients with thoracic aortic aneurysm who smokeve double the rate of aneurysm expansion (433). Aneurysmpansion and rupture after Type B dissection are not affectedcigarette smoking (434). Smoking cessation reduces the

te of MI and death in patients with noncoronary atheroscle-sis (435). Patients who smoke require close follow-up innjunction with medical and other support to achieve com-
ete smoking cessation.

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2.2. Surgical and Endovascular Treatment byocation of Disease

2.2.1. ASCENDING AORTA AND AORTIC SINUSES

2.2.1.1. Recommendations for Asymptomaticatients With Ascending Aortic AneurysmASS I

Asymptomatic patients with degenerative thoracic aneurysm,chronic aortic dissection, intramural hematoma, penetratingatherosclerotic ulcer, mycotic aneurysm, or pseudoaneurysm,who are otherwise suitable candidates and for whom theascending aorta or aortic sinus diameter is 5.5 cm or greater,should be evaluated for surgical repair. (371) (Level of Evi-dence: C)Patients with Marfan syndrome or other genetically mediateddisorders (vascular Ehlers-Danlos syndrome, Turner syndrome,bicuspid aortic valve, or familial thoracic aortic aneurysm anddissection) should undergo elective operation at smaller diam-eters (4.0 to 5.0 cm depending on the condition; see Section5) to avoid acute dissection or rupture. (81,114,143,371,436–439) (Level of Evidence: C)Patients with a growth rate of more than 0.5 cm/y in an aortathat is less than 5.5 cm in diameter should be considered foroperation. (Level of Evidence: C)Patients undergoing aortic valve repair or replacement andwho have an ascending aorta or aortic root of greater than 4.5cm should be considered for concomitant repair of the aorticroot or replacement of the ascending aorta. (Level of Evi-dence: C)

ASS IIa

Elective aortic replacement is reasonable for patients withMarfan syndrome, other genetic diseases, or bicuspid aorticvalves, when the ratio of maximal ascending or aortic root area(� r2) in cm2 divided by the patient’s height in meters exceeds10. (16,143) (Level of Evidence: C)It is reasonable for patients with Loeys-Dietz syndrome or aconfirmed TGFBR1 or TGFBR2 mutation to undergo aorticrepair when the aortic diameter reaches 4.2 cm or greater bytransesophageal echocardiogram (internal diameter) or 4.4 to4.6 cm or greater by computed tomographic imaging and/ormagnetic resonance imaging (external diameter). (78) (Levelof Evidence: C)

ortic diameter is a major criterion for recommendingective operation in asymptomatic patients with aneurysmthe thoracic and thoracoabdominal aorta. This assumesat the risk of operation is low (less than 5%). Currently,rtic diameter perpendicular to the axis of flow aseasured by CT is the dimension most often used totermine the size of the enlarged aorta. This recommen-tion is based on the observation that the risk of anverse event (rupture, dissection, death) exceeds the riskelective operation when the maximum diameter exceeds

5 to 6.0 cm (374,436,437,440) (Figure 30). Formulasat incorporate height and aortic cross-sectional area haveen developed to establish thresholds for operation in
orter patients but are less widely used (16,143). pa
The morphology and histopathology of thoracic aorticlargements affect the natural history of aortic diseases,cluding the risk of rupture or dissection, and thus canfluence the decision to intervene (Figures 31 and 32).usiform aneurysms are most common and behave in alatively predictable manner. Aortic dimension can thusused as an indication for operation. Saccular aneurysms

cur less frequently and may be associated with a greatersk of rupture. Many of these are actually pseudoaneu-sms, which can develop after previous trauma or aorticrgery, or with PAUs, and which result in focal disrup-

on or weakening of the layers of the aorta.Patients with substantial dilatation of the aortic sinusesay develop asymptomatic aortic regurgitation as a resultloss of coaptation of the otherwise normal aortic valvesps. Patients with associated bicuspid aortic valve diseaseay have asymptomatic stenosis or regurgitation of thelve. In these patients, the valvular disease may be andication for operative intervention (5).

2.2.1.2. Recommendation for Symptomatic Patientsith Thoracic Aortic Aneurysm

ASS I

Patients with symptoms suggestive of expansion of a thoracicaneurysm should be evaluated for prompt surgical interventionunless life expectancy from comorbid conditions is limited orquality of life is substantially impaired. (Level of Evidence: C)

ymptoms associated with thoracic aneurysms usuallyvelop later in the course of enlargement of the aorta andost commonly result from impingement of the aneurysm

adjacent structures. Aneurysms of the ascending aortad aortic sinuses may result in symptoms related to thertic regurgitation that develops as a result of the pro-essive aortic enlargement. Chest or back pain in theesence of an enlarged thoracic aorta is a predictor ofrtic rupture (336,441). Patients who develop an acuteype A AoD commonly present with severe chest or back

gure 30. Effect of aortic aneurysms diameter on risk of com-ication. For thoracic aortic aneurysms of all etiologies.apted from Elefteriades et al (437).

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2.2.1.3. Endovascular Grafting for Ascending Aorticneurysm. At the time of this writing, endovascular stentafts have not been approved by the US Food and Drugdministration for treatment of aneurysms or other con-tions of the ascending aorta.

2.2.1.4. Recommendations for Open Surgery forscending Aortic AneurysmASS I

Separate valve and ascending aortic replacement are recom-

gure 31. Ascending aortic aneurysm of degenerative etiology. Ctery disease; CT, computed tomographic imaging; and MR, mag

mended in patients without significant aortic root dilatation, in se

elderly patients, or in young patients with minimal dilatationwho have aortic valve disease. (Level of Evidence: C)Patients with Marfan, Loeys-Dietz, and Ehlers-Danlos syn-dromes and other patients with dilatation of the aortic root andsinuses of Valsalva should undergo excision of the sinuses incombination with a modified David reimplantation operation iftechnically feasible or, if not, root replacement with valvedgraft conduit. (134,164,442–444) (Level of Evidence: B)

scending Aortic Aneurysms: The extent of aortic re-

dicates coronary artery bypass graft surgery; CAD, coronarysonance imaging.

ABG in

ction and the need for ancillary procedures are deter-

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ined by preoperative testing and intraoperative findings.ncillary procedures that may be performed concurrentlyclude coronary artery bypass graft surgery, valve replace-ent or repair, repair of cardiac septal defects, closure ofscular fistulas, and ablative therapy for arrhythmias.For patients with isolated aneurysms confined to thecending aorta, resection and graft replacement is theost commonly performed and recommended procedure.lternatively, reduction aortoplasty with or without exter-l reinforcement has only been performed in very limited

gure 32. Ascending aortic aneurysms associated with genetic dns for Asymptomatic Patients With Ascending Aortic Aneurysmd Associated Congenital Variants in Adults (Section 6.1). CABG

sease; CT, computed tomographic imaging; and MR, magnetic r

rcumstances (445,446). pa

For patients with aortic valve stenosis who require valveplacement, the choice of valve substitute is determinedage of the patient, presence of comorbid disease, risk of

mplications related to anticoagulation and reoperation,d life expectancy (139).For patients with aortic regurgitation associated with acuspid aortic valve, repair of the aortic valve with orithout root remodeling or tailoring of the sinotubularnction is preferable if the valve is not severely fibrotic orlcified (99,140). For patients with a dilated aortic root,

. *Depends on specific genetic condition. †See Recommenda-n 9.2.2.1.1) and Recommendations for Bicuspid Aortic Valvees coronary artery bypass graft surgery; CAD, coronary arteryce imaging.

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lve grafts containing either mechanical or biologicallves are implanted.Ascending aneurysms larger than 4.5 to 5.0 cm requirepair or tube graft replacement when aortic valve repair orplacement is the primary indication for operation (5). Inderly patients, ascending aortic aortoplasty when thertic diameter does not exceed 5.0 cm may be anceptable alternative.Aortic Valve and Root: In patients with aortic valvegurgitation and root dilatation, aortic valve repair andot-sparing procedure may be the primary procedure. Intients with Marfan syndrome or with tricuspid aorticlve regurgitation, a modification of the David reimplan-tion operation may be considered. (94,95,97–99,447)omposite valve grafts with either biological or mechan-al valves are an alternative option, particularly for valvularenosis (99,140,441,448).

2.2.2. RECOMMENDATIONS FOR AORTIC ARCH ANEURYSMS

ASS IIa

For thoracic aortic aneurysms also involving the proximalaortic arch, partial arch replacement together with ascendingaorta repair using right subclavian/axillary artery inflow andhypothermic circulatory arrest is reasonable. (222,449,450)(Level of Evidence: B)Replacement of the entire aortic arch is reasonable for acutedissection when the arch is aneurysmal or there is extensiveaortic arch destruction and leakage. (222,450) (Level ofEvidence: B)Replacement of the entire aortic arch is reasonable for aneu-rysms of the entire arch, for chronic dissection when the archis enlarged, and for distal arch aneurysms that also involve theproximal descending thoracic aorta, usually with the elephanttrunk procedure. (451–453) (Level of Evidence: B)For patients with low operative risk in whom an isolateddegenerative or atherosclerotic aneurysm of the aortic arch ispresent, operative treatment is reasonable for asymptomaticpatients when the diameter of the arch exceeds 5.5 cm. (374)(Level of Evidence: B)For patients with isolated aortic arch aneurysms less than 4.0cm in diameter, it is reasonable to reimage using computedtomographic imaging or magnetic resonance imaging, at 12-month intervals, to detect enlargement of the aneurysm. (Levelof Evidence: C)For patients with isolated aortic arch aneurysms 4.0 cm orgreater in diameter, it is reasonable to reimage using com-puted tomographic imaging or magnetic resonance imaging, at6-month intervals, to detect enlargement of the aneurysm.(Level of Evidence: C)

neurysms of the aortic arch are commonly associated witheurysmal disease or dissection of the ascending aorta or thejacent descending thoracic aorta, and the indications forerative intervention in these patients are those for thejacent aortic segment. This relates to the need for hypo-ermic cardiopulmonary bypass and an interval of hypother-ic circulatory arrest, and to higher operative mortality and
roke rates than those observed following operation for
olated aneurysms of the ascending or descending thoracicrta (451–459). As with ascending aneurysms, a growth ratemore than 0.5 cm/y in the absence of symptoms could bensidered an indication for operation.

Symptoms associated with aortic arch aneurysms such asarseness resulting from stretching of the left recurrent

ryngeal nerve, dysphagia, dyspnea, and chest or back paine indications for operative intervention for patients withch aneurysms unless life expectancy is quite limited.itability for operative intervention involves similar risksessment to that for aneurysm or other disorders of thecending aorta and aortic root.

The innominate, left carotid, and left subclavian arteriesay require separate grafting. For short periods of circulatoryrest, the use of retrograde or antegrade brain perfusion hast conclusively been shown to add further brain protection;wever, use of the subclavian or axillary artery bypass withside graft reduces the risk of stroke (449).

2.2.2.1. Open Surgery. At present, endovascular stentafts have not been approved by the US Food and Drugdministration for treatment of aneurysms or other con-tions of the aortic arch. For patients with large aneu-sms who are not candidates for conventional openeration, experience is accumulating with operative pro-dures that involve translocation of the brachiocephalicteries from the aortic arch using branch grafts from theoximal ascending aorta, and placement of an endovas-lar graft into the distal ascending aorta, the entire aorticch, and a segment of the adjacent descending thoracicrta (371,460,461).Cardiopulmonary bypass with some degree of hypother-ia is required for operations that require replacement ofe aortic arch. Brain protection can be achieved byofound hypothermia alone, direct antegrade perfusion ofor more of the brachiocephalic arteries, or retrograderfusion using cold oxygenated blood that is infused intoe superior vena cava during the arrest period11,449,462–467) (see Section 14.5.1). The aortic arch isplaced with a synthetic graft. The brachiocephalic arter-s are attached to the graft using a patch of the aortahich contains the origins of the 3 vessels or separatelying a graft that contains 3 branches. The proximal andstal ends of the aortic graft are attached to normalgments of ascending and descending thoracic aorta.An “elephant trunk” procedure has been used to recon-

ruct the arch and then provide a Dacron graft landingne for endovascular stent graft treatment of descendingoracic aortic aneurysms (Figure 33).

2.2.3. DESCENDING THORACIC AORTA AND

ORACOABDOMINAL AORTA

2.2.3.1. Recommendations for Descending Thoracicorta and Thoracoabdominal Aortic AneurysmsASS I

For patients with chronic dissection, particularly if associated
with a connective tissue disorder, but without significant

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comorbid disease, and a descending thoracic aortic diameterexceeding 5.5 cm, open repair is recommended. (371,382,468) (Level of Evidence: B)For patients with degenerative or traumatic aneurysms of thedescending thoracic aorta exceeding 5.5 cm, saccular aneu-rysms, or postoperative pseudoaneurysms, endovascular stentgrafting should be strongly considered when feasible.(371,469) (Level of Evidence: B)For patients with thoracoabdominal aneurysms, in whom endo-vascular stent graft options are limited and surgical morbidityis elevated, elective surgery is recommended if the aorticdiameter exceeds 6.0 cm, or less if a connective tissuedisorder such as Marfan or Loeys-Dietz syndrome is present.(371) (Level of Evidence: C)For patients with thoracoabdominal aneurysms and with end-organ ischemia or significant stenosis from atheroscleroticvisceral artery disease, an additional revascularization proce-dure is recommended. (470) (Level of Evidence: B)

t the time of publication of this document, 3 endovas-lar stent grafts have been approved by the US Food andrug Administration only for aneurysms involving thescending thoracic aorta. Although the feasibility andfety of endovascular stent grafting of the descendingrta have been demonstrated for other pathologic condi-

ons including acute and chronic Type B AoD, IMH,AU, acute traumatic aortic transsection, and pseudoan-rysms, these conditions are currently considered “offbel.”There are no published randomized trials that comparee outcomes of endovascular stent grafting with conven-

onal open operation or nonoperative management. Thus,
commendations for use are based principally on obser- pa
tional studies and nonrandomized comparisons of co-orts of patients.

2.2.3.2. Endovascular Versus Open Surgical Ap-roach. The potential advantages of endovascular graft-g over open operation include the absence of a thora-tomy incision and the need for partial or totaltracorporeal circulatory support and clamping of therta, as well as lower hospital morbidity rates and shorterngth of hospital stay.Endovascular grafting may be of particular value intients with significant comorbid conditions (older age,bstantial cardiac, pulmonary and renal dysfunction) whoould be considered poor or noncandidates for openrgery. Patients who are not considered candidates foren surgery but who have undergone endovascular graft-g have substantially poorer long-term outcomes thantients who are reasonable candidates for open operationd are treated with endografts (471). Furthermore, inter-ntion (endovascular stent graft or open surgical graftplacement) for a descending aneurysm has real risks ofortality and morbidity, including the risk of spinal cordchemic injury. All physicians should work collaboratively

ong specialities during the initial decision-making stepsdetermine via consensus whether a particular patient’sthology, risk factors, and projected natural history if

eated medically justify an intervention on the descendingoracic aorta, either a stent graft or an open procedure.There are no data that conclusively demonstrate that theevalence of spinal cord ischemic injury (lower extremity

Figure 33. Elephant trunk procedure. Left,Preoperative disease. Middle, Stage I withreplacement of the ascending aorta and archwith a Dacron graft with the distal graft su-tured circumferentially to the aorta distal tothe left subclavian artery and the free end ofthe graft (“elephant trunk”) within the de-scending aneurysm. Right, Completion of theprocedure using an endovascular stent graftattached proximally to the “elephant trunk”and the distal end secured to a Dacron graftcuff. Images reprinted with permission fromthe Cleveland Clinic Foundation.

ralysis or paresis) is less for endovascular approaches

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an for open surgical repair. Similarly, there are no firmta to indicate that overall costs of medical care are lower

ith endovascular procedures. Although the costs of theitial hospitalization may be lower because of reducederative time and a shorter length of stay, these benefitsay be negated by the cost of the devices, the need forbsequent interventions, and the cost and dissatisfactionrepeated imaging studies, which are necessary in thestoperative period (472,473).Some patients are not suitable candidates for endovas-lar grafting procedures. Absence of suitable “landingnes” above and below the aneurysm (usually 2 to 3 cm ofrmal diameter aorta without circumferential thrombus)a contraindication. A width of the aorta at the landingnes that exceeds the recommended width for the largestailable endovascular grafts (generally 10% to 15% largeran the width of the aorta) is also a contraindication.Lack of vascular access sites to insert the relatively

rge-bore sheaths that are necessary for deployment of theafts is also a contraindication. Severe atherosclerosis andtraluminal thrombus of the aorta may increase the risk ofbolic stroke during manipulation of guidewires and

theters and represents a relative contraindication (474).

2.2.3.3. End-Organ Preservation During Thoracicndograft Implantation. Because aneurysmal diseasen involve any portion of the aorta, organ preservationring repair of either aneurysmal disease or dissection isimportant part of the implant procedure. Aneurysms

volving the aortic arch pose a significant risk to cerebrald upper extremity blood flow with endovascular repair.

he need to cover either the left common carotid or thenominate artery to treat arch aneurysmal disease isfrequent. Intentional coverage of the left subclaviantery is more common, occurring in approximately 50% oforacic endograft implants. Most patients tolerate cover-e of the left subclavian artery without upper extremity

chemia (475–478), but recently several groups haveggested that these patients may be at higher risk ofrioperative stroke and spinal cord ischemic injury (479–1). In the Talent VALOR trial, the need for intentionaleimplant left subclavian artery bypass was only 5.2%82). To minimize the risk associated with intentionalverage of the left subclavian artery, it is recommendedat the patency of the contralateral right subclavian andrtebral arteries be determined preoperatively by CT,R, or invasive angiography. Additionally, verificationat the vertebral arteries communicate at the basilar artery

either transcranial Doppler or angiography is alsocommended. If these steps are taken to ensure that thentralateral posterior circulation is intact, the need torform a left subclavian artery bypass postoperatively toevent symptomatic arm claudication or vertebral basilarsufficiency is infrequent.Spinal cord ischemia leading to paralysis is one of therious potential complications of the thoracic endograft
ocedure. Intercostal arteries are intentionally covered by co
e endograft. There is evidence that the risk of spinal cordchemia may be greater when treatment involves coverage

most of the descending thoracic aorta (e.g., from leftbclavian to celiac artery) (483). Additionally, patientsho have had previous repair of abdominal aneurysmither open or endovascular) are at increased risk forinal cord ischemia and paraplegia (484). In 1 study, the

sk of spinal cord ischemia was 10% to 12% in patientsith previous abdominal aneurysm repairs and 2% intients who did not have previous abdominal aneurysmpairs (485). In circumstances where it is necessary tover most of the descending thoracic aorta or if thetient has had a previous abdominal aneurysm repair,rebrospinal fluid (CSF) pressure monitoring and drain-e are an important strategy to minimize the risk ofraplegia (486).Treatment of TAAs with an endograft may requiretentional coverage of the celiac and/or superior mesen-ric arteries to achieve a seal at the distal attachment site.

these instances, a superior mesenteric-to-celiac arterypass graft or an aorta-to-superior mesenteric and/orliac artery bypass graft has been used as a first-stagebranching procedure before proceeding with the en-graft implant (Figure 34). However, risk of the de-anching operation may be no lower than conventionalen repair, and therefore these operative approaches arerformed selectively (487). Fenestrated grafts are invelopment and are undergoing clinical trials. Roselli et(488) published initial results in the first 73 patientsdergoing in situ endovascular repair of extensive TAAsing a branch endograft with promising results in a

igh-risk population. With careful attention to preserva-on of blood flow to the mesenteric vessels, the incidencemesenteric ischemia with endograft implants is approx-ately 3% (482). Most instances of mesenteric ischemia

e the result of emboli rather than malperfusion owing toverage or occlusion of a mesenteric vessel.AoDs pose a complex situation for intestinal, renal, orwer extremity perfusion, as the branches of the aorta ine abdominal cavity may be perfused from either the truefalse lumen. Often, both the true and false lumens aretent and some of the visceral, renal, or lower extremityssels are fed by one channel and the remainder by theher. Thus, the operator must consider how blood flowaches vital organs in the abdominal cavity before con-dering treatment of an AoD with an endograft. In mostses, where descending thoracic dissections are treatedith an endograft, the important treatment principle is tover the proximal entry tear obliterating the false lumen.owever, if the false lumen supplies blood flow to thesceral vessels, blood flow to these organs may be com-omised after endograft implantation. Stenting of thessels at risk from the true lumen or establishing flowmmunication between true and false lumen more dis-lly with a fenestration procedure may prevent such
mpromise. In cases of acute Type B AoD treated with

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dografts, coverage of the proximal entry site into thelse lumen may result in healing of the tear downstreamith restoration of blood flow from the true lumenithout the need for adjunctive fenestration procedures90).

2.2.3.4. Periprocedural Complications of Endograftrocedures. The acute complications of thoracic en-graft procedures are related to problems with access and

ith device implantation. Vascular access is a substantialsue with thoracic endograft procedures. The sheathquired to deploy endovascular grafts is size 20 French oreater and can be up to size 25 French. Many patients

ave femoral arteries that are too small to accommodatech large sheaths. Use of the iliac artery or the aorta forcess is required in approximately 15% of the patients.fection at the access site is not an infrequent complica-

on. Bleeding complications are less frequent. Averageood loss in the Talent VALOR trial was 371 mL, ande need for transfusion in the STARZ TX2 clinical trialtudy results on the Zenith TX2 Endovascular Graft for

gure 34. Schema of TAA treated with initial left iliac artery–to–ft renal artery–to–superior mesenteric artery bypass graft andbsequent placement of a thoracoabdominal endograft. Proxi-al superior mesenteric artery and left renal arteries were li-ted. SMA indicates superior mesenteric artery; and TAA, tho-coabdominal aneurysm. Adapted from Flye et al (489).

e Thoracic Aortic Aneurysms) was 3% (469,482). ba

hromboemboli to mesenteric, renal, or lower extremityssels can also occur (491).Major adverse events related to the device occur in 10%12% of patients in the initial 30-day perioperative

riod. Stroke was observed in 2.5% of the patients in theTARZ TX2 clinical trial (469) and 3.6% in the TalentALOR trial (482). It has been reported to be as high as

in other studies (474,492). Most strokes occur as asult of atheroemboli from the transverse aortic arch withanipulation of the guidewires and device in the archssels (474). Paraplegia varied from 1.3% in the STARZX2 clinical trial to 1.5% in the Talent VALOR trial to

in the TAG trial (469,482,493).Cardiac complications, principally MI, occur rarely (2%4%) (469,482). Cardiac tamponade or rupture is rare

d may be caused by either the stiff guidewires that arequired to deliver the devices or excessive afterloadeated by the balloons that are inflated to produce a sealthe graft to the aortic wall. Ventricular tachycardia or

ntricular fibrillation has been reported (469). Pulmonarymplications include postoperative pneumonias, whichcur in less than 5% of patients. Acute renal failurequiring dialysis is also uncommon, reported to occur in3% of patients in the Talent VALOR trial (482). Deviceplant infection is exceedingly rare and has been ob-

rved mainly in situations where the device was implantedan infected medium emergently (i.e., mycotic aneu-

sms or aortoenteric fistulae) (482).Endoleak is defined as the persistence of blood flowtside the lumen of the endoluminal graft but within theeurysm sac, based on imaging. Endoleaks are classified

ble 14. Classification of Endoleaks

pe Cause of Perigraft Flow Sequelae and Treatment Strategy

Inadequate seal atproximal and/or distalgraft attachment site

Systemic arterial pressure transmitted tothe aneurysm sac, leading to expansionand rupture. Should be repaired when

diagnosed.

Retrograde aorticbranch arterial blood

flow into aneurysm sac

May spontaneously thrombose. Ifaneurysm is stable or shrinking,

observe. If aneurysm is expanding,embolic occlusion of branch artery

indicated but often difficult.

Structural failure ofendograft (e.g., stent

graft fractures, holes infabric, junctional

separations)

Systemic arterial pressure transmitted tothe aneurysm sac, leading to expansionand rupture. Should be repaired when

diagnosed.

Stent graft fabricporosity

Noted at time of implantation andusually resolved with reversal of

anticoagulation.

Expansion of aneurysmwithout demonstrableendoleak, also called

“endotension”

Reline the endograft with a secondendograft.

Adapted from Veith et al (494).

sed on the source of blood flow (Table 14).

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Perforation or dissection of the aorta at the implanta-on site is infrequent but usually reported with stentstems that have uncovered or bare proximal attachmentents (495–499). Involution or infolding of the en-grafts may occur and has been most frequently reported

ith grafts applied to a relatively small aorta whereersizing can be substantial, especially in trauma cases66,500,501). Infoldings can also occur when there isadequate conformity of the device to the aortic wall in agion of marked curvature or “beaking” (see Figure 27).his leads to collapse of the endograft, gross endoleak, ande potential for acute occlusion of flow to the descendingoracic aorta. It can be prevented (or managed after itcurs) with implantation of a reinforcing stainless steelent at the proximal leading edge of the implant (500).Late complications of thoracic aortic endografting in-

ude endoleaks, continued aneurysm growth, metal fa-gue and stent fracture and kinking, detachment, migra-on, perforation, and infection of the implanted device.ndoleaks occur in 10% to 20% of patients (482). Theequency is greatest in the first month following implan-tion and declines over the ensuing 5 years (502). Theost frequent type of endoleak is Type IA, or proximaltachment site failure (see Section 8.6) (469,482,503–5). Migration occurs infrequently—at 1-year follow-up0.4% of cases in the TAG clinical trial and 3.9% of casesthe Talent VALOR trial (469,482,506).Late perforations of the aorta by the endograft can occurd are common with the use of oversized grafts and/orafts with bare or uncovered stents (507,508). Most latevice complications such as endoleak and migration are

eatable with implantation of graft extensions. The overalled for repeat interventions to maintain endograft integ-

ty is 6% to 7%, most commonly in the form of implan-tion of an extension cuff. Conversions to open operatione rarely needed (1% to 2%) (476,480,505,509,510). Latemplications (9 to 24 months) include stent fractures,hich are often asymptomatic (511). There are caseports of fractures leading to endograft dysfunction,doleak, migration, and/or embolization (512). En-graft infection, usually as a result of an ongoing systemicfection, or as a result of infected structures adjacent toe graft (513,514), is uncommon, but when it occurs, it isry difficult to eradicate without explantation of the deviced, without explantation, can lead to aortic rupture (515).The experience with the use of endografts for the

eatment of acute descending AoD is limited. A meta-alysis of 609 published cases suggests that when en-grafts are used, mortality is slightly higher (5.3%) and

te conversions to open procedures are more frequent.5%) than data reported for treatment of aneurysms. Thesk of major complications including stroke, paraplegia,d aneurysm rupture appears to be similar in bothnditions (516). A complication unique to endografteatment of acute Type B dissections is conversion of the
ssection to a retrograde Type A dissection, creating a vi
rgical emergency (480,517). Until and unless this life-reatening complication is understood and eliminated,dograft treatment of acute Type B dissections should bedertaken at institutions with a team qualified to performen aortic surgery.In the absence of Level A or Level B evidence regardinge relative efficacy of open and endovascular proceduresr treatment of descending aortic aneurysms, no firmcommendations can be made regarding the optimalethod of treatment. Among comparable patients treatedith either open or endovascular procedures, the midtermsults can be anticipated to be equivalent. The earlyortality advantage of endovascular procedures may best during follow-up such as that seen with endovasculareatment of AAAs (518,519). The long-term durability ofdovascular stent grafts is uncertain; currently, availableafts may have a durability of no more than 10 years.ounger patient age may be a relative contraindication todografting.There are no data to indicate that endovascular stentafting should be performed in patients with asymptom-ic descending aortic aneurysms that are less than 5.5 cmdiameter, because the risk of operation (approximately) exceeds the risk of rupture or dissection (371) (Table). Undoubtedly, as new iterations of devices are intro-ced, these guidelines will change.

2.2.3.5. Open Surgical. Diseases of the aorta thatquire extensive thoracoabdominal surgical or interven-onal approaches fall into 3 large groups: 1) degenerativeeurysms, 2) AoD resulting in subsequent aneurysms or

ble 15. Summary of Society of Thoracic Surgeonscommendations for Thoracic Stent Graft Insertion

tity/Subgroup ClassificationLevel ofEvidence

netrating ulcer/intramural hematoma

Asymptomatic III C

Symptomatic IIa C

ute traumatic I B

ronic traumatic IIa C

ute Type B dissection

Ischemia I A

No ischemia IIb C

bacute dissection IIb B

ronic dissection IIb B

generative descending

�5.5 cm, comorbidity IIa B

�5.5 cm, no comorbidity IIb C

�5.5 cm III C

ch

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oracoabdominal/severe comorbidity IIb C

Reprinted from Svensson et al (371).

sceral ischemia, and 3) occlusive disease of the visceral

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teries. Selection of patients for repair is based onmptoms, risk of death from rupture, and end-organchemia, provided associated comorbidity does not pre-nt surgical repair. In patients with lower chest or upperdominal pain, CT or MR is performed to determine ife patient has a contained rupture, leak, or an aneurysmpinging on surrounding structures that may be causingin. The perioperative risk of death is approximately 80%

ith emergency surgery; a few patients will recover with-t a major complication limiting quality of life, and few

ill be long-term survivors because of comorbid disease. Intients with pain from compression of surrounding struc-res, if comorbid disease is not a factor, results arensiderably better with a 10% to 20% risk of death. Intients undergoing elective surgery, the risk of death is

to 10%, depending on the extent of repair.The extent of repair for TAA is classified by the

rawford types: Type I is a repair that extends from theoximal descending aorta above T6 to the renal arteries;ype II, the highest risk group, extends from the proximalscending aorta above T6 to below the renal arteries;ype III extends from the distal descending aorta below6 to below the diaphragm for variable extents; and Type

extends from the diaphragm and involves most of thedominal aorta. This classification correlates with the riskparalysis, renal failure, and death.Open surgical repair of TAA improved over the past 15ars, particularly on preventing postoperative paralysis.istorically, one of the most serious complications wasralysis, either paraparesis or paraplegia, caused by spinalrd injury whose risk is for Type I TAA repair, 15%; forype II, 30%; Type III, 7%; and for Type IV, 4% (381).urrent risk of paralysis is 3% to 5%, depending somewhat

the extent of repair (381,382,520–526).The risk of renal failure may be reduced by preoperativey admission, fluid hydration starting the day before surgery,d the addition of bicarbonate to the hydration regimen andpothermia protection during the operative procedure.

2.2.3.6. End-Organ Preservation During Open Tho-coabdominal Repairs. For thoracoabdominal aortic re-irs done in combination with proximal repairs of thertic arch, the key vital end organs to protect are the heartd brain. Flooding the field with CO2 appears to beneficial (527,528). When the aortic arch requires repair,

ypothermic circulatory arrest is usually required. Protec-on of the brain involves ensuring that calcium plaques orheromata are not disturbed to prevent brain emboliza-on. The temperature and where it is best measured, athich circulatory arrest is commenced, are debated butost large series have recommended circulatory arrest at amperature below 20°C (211).Perfusion of the celiac artery or superior mesenterictery does not appear to be needed to protect thedominal organs in most patients where moderate hypo-ermia (30° to 32°C) is used. Protection of the left lung
ring left thoracotomy repairs is important to avoid ju
cerations and bleeding into the parenchyma. Deflation ofe left lung may be of benefit. However, disruption of thesceral pleura with attendant complications of bleedingd air leak may be unavoidable, particularly if adhesionse present due to prior surgery or inflammatory changes.

2.2.3.7. Aortic Dissection With Malperfusion. Re-l, mesenteric, lower extremity, or cord malperfusioncompanies up to one third of acute AoD and roughlyubles mortality (248,529,530). In the case of Type A

oD with malperfusion, there is controversy over whethertient outcomes are improved by first repairing the aortad then treating persistent malperfusion (531) or by firstrrecting the malperfusion and then repairing the aorta47,532). The general consensus is to first repair therta, because this will correct malperfusion in mosttients. In the case of Type B AoD, there is a generalnsensus that medical management should be supple-ented by open or endovascular intervention when thereevidence for renal, mesenteric, lower extremity, or cordalperfusion (248,490,533).

0. Special Considerations in Pregnantatients With Aortic Disease

0.1. Effects of Pregnancy on the Aortaysiologic effects of pregnancy may have a profound effectt only on aortic stress but potentially on arterial ultra

ructure as well. The pregnant state is characterized bycreases in maternal blood volume, heart rate, blood pres-re, stroke volume, and cardiac output (534,535). Takengether, the combined effects lead to greater arterial wallnsion as well as intimal shear forces. These changes beginthe first and second trimesters but are most notable in the

ird trimester and peripartum period. Whether arterial walleakening itself occurs during pregnancy remains controver-al. Arterial dissection and/or rupture may occur with theghest incidence in the third trimester (approximately 50%)d peripartum period (33%). In one of the few prospective

udies of pregnant patients with Marfan syndrome, 4.4% ofrefully monitored patients developed aortic dissection34). In unmonitored patients, the risk is likely higher.

0.2. Epidemiology of Chronic and Acuteortic Conditions in Pregnancyarfan syndrome, Ehlers-Danlos syndrome, and other non-arfan aortic disease may manifest during pregnancy. Al-ough clearly rare, it has been estimated that half of AoDd/or ruptures in women younger than 40 years of age haveen associated with pregnancy (536). Most dissections occurthe ascending aorta, although dissection or rupture of

rtually any artery in the body has been described. Indition, pregnancy-related expansion of the sinotubular
nction may lead to aortic valve insufficiency.

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0.3. Counseling and Management of Chronicortic Diseases in Pregnancy

.3.1. Recommendations for Counseling andanagement of Chronic Aortic Diseases in Pregnancy

ASS I

Women with Marfan syndrome and aortic dilatation, as well aspatients without Marfan syndrome who have known aorticdisease, should be counseled about the risk of aortic dissec-tion as well as the heritable nature of the disease prior topregnancy. (74,91) (Level of Evidence: C)For pregnant women with known thoracic aortic dilatation or afamilial or genetic predisposition for aortic dissection, strictblood pressure control, specifically to prevent Stage II hyper-tension, is recommended. (Level of Evidence: C)For all pregnant women with known aortic root or ascendingaortic dilatation, monthly or bimonthly echocardiographic mea-surements of the ascending aortic dimensions are recom-mended to detect aortic expansion until birth. (Level of Evi-dence: C)For imaging of pregnant women with aortic arch, descending,or abdominal aortic dilatation, magnetic resonance imaging(without gadolinium) is recommended over computed tomo-graphic imaging to avoid exposing both the mother and fetusto ionizing radiation. Transesophageal echocardiogram is anoption for imaging of the thoracic aorta. (Level of Evidence: C)Pregnant women with aortic aneurysms should be deliveredwhere cardiothoracic surgery is available. (Level of Evi-dence: C)

ASS IIa

Fetal delivery via cesarean section is reasonable for patientswith significant aortic enlargement, dissection, or severeaortic valve regurgitation. (91) (Level of Evidence: C)

ASS IIb

If progressive aortic dilatation and/or advancing aortic valveregurgitation are documented, prophylactic surgery may beconsidered. (537) (Level of Evidence: C)

this regard, risk of major aortic complications duringegnancy appears to be low when the aortic root diameter isss than 4.0 cm (538). Such individuals may have one orore successful pregnancies. For patients with an aorticameter greater than 4.0 cm and Marfan syndrome, half willve come to prophylactic surgery during pregnancy, willve a rupture, or will have life-threatening growth. Optimaleventive therapy in the pregnant woman with known aorticsease includes use of beta blockers to control heart rate andduce shear stresses, particularly in the third trimester andripartum period. Both angiotensin-converting enzyme in-bitors and angiotensin receptor blockers are contraindicatedring pregnancy.

0.4. Evaluation and Management of Acuteortic Syndromes During Pregnancyegnant women with Marfan syndrome, bicuspid aorticlve and ascending aneurysms, Ehlers-Danlos syndrome,d non-Marfan familial thoracic aortic aneurysm and dis-
ction may present with acute aortic syndromes at any point pr
ring the pregnancy but are particularly prone to do so in thest trimester, during delivery, or in the early postpartumriod. Such women may have no knowledge of theirderlying aortic condition until presentation with their acutertic problem.Obviously, acute AoD poses a huge risk for both theother and the unborn child. Optimal treatment parallels thatnonpregnant women but with the added complication of

hen and how to deliver the child. Optimal care includesvolvement with a high-risk maternal-fetal team along with

aortic specialty team capable of medical, percutaneous,d surgical aortic treatment. For Type A AoD occurringring the first or second trimester, urgent surgical repair withgressive fetal monitoring is preferred. Fetal loss duringpothermia and cardiopulmonary bypass is common. Whenssection complicates the third trimester, urgent cesareanction followed by aortic repair appears to offer the bestance for survival for the unborn child and the mother. Forute arch or Type B AoD, medical therapy is preferredless percutaneous stent grafting or open surgery is man-ted by malperfusion, aortic rupture, or subacute aorticaking (539).

1. Aortic Arch and Thoracic Aortictheroma and Atheroembolic Disease

1.1. Recommendations for Aortic Archnd Thoracic Aortic Atheroma andtheroembolic DiseaseASS IIa

Treatment with a statin is a reasonable option for patients withaortic arch atheroma to reduce the risk of stroke. (540) (Levelof Evidence: C)

ASS IIb

Oral anticoagulation therapy with warfarin (INR 2.0 to 3.0) orantiplatelet therapy may be considered in stroke patients withaortic arch atheroma 4.0 mm or greater to prevent recurrentstroke. (Level of Evidence: C)

1.2. Clinical Descriptionortic arch atheroma is a risk factor for ischemic stroke based

autopsy (541,542), TEE (543–548), and intraoperativetrasonographic studies (549) (Figure 35). In particular,aques 4 mm or greater in thickness proximal to the origin ofe left subclavian artery are associated with stroke andnstitute one third of patients with otherwise unexplained

roke (542). These patients, even on antiplatelet therapy,rry a risk of recurrent ischemic stroke as high as 11% at 1ar, and the risk of a new vascular event (ischemic stroke,I, peripheral event, and vascular death) is 20%, 36%, and% at 1, 2, and 3 years, respectively (550). The RR of new

chemic stroke was 3.8 (95% CI 1.8 to 7.8, P�0.002), andat of new vascular events was 3.5 (95% CI 2.1 to 5.9,�0.001), independent of carotid stenosis, atrial fibrillation,ripheral artery disease, or other risk factors (550). Other

udies showed that aortic arch plaques were independent
edictors of recurrent strokes, MI and vascular death

(5ri

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51–553). Patients with noncalcified plaques were at highersk for recurrent vascular events (554).Regarding the natural history of aortic arch atheroma, Senal (555) noted progression in 29% and regression (definedan increase or decrease in plaque thickness by 1 grade or

eater, respectively) in 9%. Montgomery et al (556) reportedpatients with moderate-to-severe aortic plaque noted on

itial bi/multiplanar TEE as part of a workup for cardiac orembolic event. Over a mean of 1 year, progression was

ported in 23% and regression in 10%. Pistavos et al (557)ed monoplanar TEE in 16 patients with familial hypercho-sterolemia taking pravastatin to show progression in 19%d regression in 38% over 2 years. Geraci and Weinberger58), using supraclavicular B-mode ultrasonography of theoximal aortic arch in 89 patients evaluated for transienturologic symptom, noted a progression rate of 19% and agression rate of 18% over a mean of 7.7 months (range 3 to

months). Sen et al (559) confirmed that in patients withroke/TIA, aortic arch atheroma progression over 12 monthsassociated with more vascular events.

1.3. Risk Factorsisk factors for the development of aortic atheroma includee, sex, heredity, hypertension, diabetes mellitus, hyperlip-emia, sedentary lifestyle, smoking, and endothelial dys-nction. Other factors include elevated levels of inflamma-ry markers (i.e., serum C-reactive protein), homocysteine,lipoprotein (560,561). Risk factors for embolic complica-ns include inflammation, shear forces of hypertension,

aque hemorrhage aneurysm formation, and iatrogenic ma-pulation. The likelihood of embolization is also increasedith complex aortic plaque, defined as plaque that containsobile thrombi or ulcerations or is 4 mm or greater inickness (562). Plaques with a larger lipid core, a predomi-nce of macrophages, a thin fibrous cap, and a lack oflcification are more “vulnerable” to disruption or rupture.alcified plaques are more stable and less likely to result in

gure 35. Ultrasound image of aortic atheroma.

bolic syndromes (562,564–565). ag

1.4. Diagnosisethods of imaging the aortic arch to detect and/or measureaque include:Transesophageal Echocardiography. TEE can provideformation of plaque mobility, ulceration, and composition66), as well as details on the anatomic relationship of theaque to the origin of the great vessels (69) with excellentterobserver and intraobserver reliability (567). LimitationsTEE in patients with stroke include the need for conscious

dation, patient cooperation for swallowing the probe, andsk of structural damage (566). A small portion of thecending aorta is masked by the tracheal air column near theigin of the innominate artery, with an estimated 2% ofaques being missed (568). Multiplanar probes may reduceacheal shadowing seen with monoplanar and biplanarobes (569).Transthoracic Echocardiography. TTE can usually im-e the aortic root and proximal ascending aorta but cannotequately assess aortic arch plaque (570,571).Epiaortic Ultrasonography. Epiaortic imaging is usefuldetect aortic arch plaque in the operative setting when the

ansducer may be placed directly over the aortic arch. Theformation may be used to select operative techniques suchoff-pump coronary artery bypass grafting to avoid cannu-

tion or cross-clamping of the aorta and reduce risk ofrioperative strokes (572,573).Contrast Aortography. The risk of invasive angiographyd the need for contrast injection and radiation makentrast aortography less useful to assess aortic arch plaque intients with stroke (574).Magnetic Resonance Imaging. MR has been validatedainst TEE for detection and measurement of aortic archaque (80% overall agreement) (568,575). It has limited usepatients who are obese, have metallic implants, or are

austrophobic. Contrast MR angiography underestimates theaque thickness but can identify morphologic features in-uding calcification, fibrocellular tissue, lipid, thrombus, andatures used to detect plaque stability and may be used toonitor aortic arch plaque progression and regression (572).Computed Tomography. CT can reliably detect andeasure protruding aortic arch plaques (568,576) and is thest of choice for detecting vascular calcification. Nonen-nced dual-helical CT may underestimate the amount ofncalcified plaque and mobile thrombus that presumably ishigh risk for embolization (568). In conjunction with

sitron emission tomography it can be used to localizeorodeoxyglucose uptake by the plaque, identifying active

aques and unstable plaques (577), but its clinical utility hast to be established.

1.5. Treatmenthere is no definitive therapeutic regimen for this high-risktient group because no randomized trial has been com-eted.

.5.1. Anticoagulation Versus Antiplatelet Therapyobile aortic atheroma have been noted to disappear duringticoagulant therapy (546) or with the use of a thrombolytic
ent (578). However, there is concern about the use of

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arfarin in patients with aortic atheroma because of theeoretical risk of plaque hemorrhage resulting in atheroem-li syndrome (i.e., blue toes, renal failure, intestinal infarc-n) (579). Anticoagulation has been associated with wors-ing (580,581), as well as improvement of an aorticrombus on anticoagulation in a patient with the atheroem-li syndrome (582). Cholesterol emboli have been docu-ented on skin, muscle, and renal biopsy samples in patientsith aortic arch atheroma seen on TEE (553,583). However,e risk of clinical atheroemboli syndrome during warfarinerapy in such patients appears to be low (only 1 episode in4 patients according to the SPAF [Stroke Prevention in

trial Fibrillation] trial) (562).Three reports shed light on the potential benefit of warfarinpatients with aortic arch atheroma. The first described 31tients with mobile lesions in the aorta on TEE (585) wherehigher incidence of vascular events was seen in patientsho were not treated with warfarin compared with thoseeated with warfarin (at the discretion of the referringysicians) (45% versus 5%). In the SPAF randomized trialpatients with “high-risk” nonvalvular atrial fibrillation, the

sk of stroke at 1 year in 134 patients with complex aorticaque was found to be reduced from 15.8% (11 events) inose treated with fixed low-dose warfarin plus aspirin (INR2 to 1.5) to only 4% (3 events) in those treated withjusted-dose warfarin (INR 2.0 to 3.0), a 75% RR reduction�0.02) for patients with atheromas who received “thera-utic range” anticoagulation (562). A third observational

udy reported on 129 patients with aortic arch atheroma onEE performed to look for a source of cerebral or peripheral

bolization (586). Treatment with oral anticoagulation,pirin, or ticlopidine was not randomly assigned. There wassignificant reduction in the number of embolic events intients with plaques greater than or equal to 4 mm whoceived oral anticoagulants (0 events in 27 patients versus 5ents in 23 patients treated with antiplatelet agents) (OR06, 95% CI 0.003 to 1.2, P�0.016). For patients withobile lesions, there was a significant reduction in mortalityhile on anticoagulants, although the trend toward fewerbolic events did not reach statistical significance in this group.These 3 reports suggest that warfarin is not harmful intients with aortic arch atheroma and may reduce stroketes. However, these studies are not randomized trials ofeatment for patients with atheromas, and the numbers arelatively small. The current ARCH (Aortic Arch Relatederebral Hazard) trial is an open-label trial where patientsith aortic arch atheroma (4 mm or greater) and nondisablingroke are being assigned to oral anticoagulation (target INR0 to 3.0) versus aspirin (75 mg/d) plus clopidogrel (75g/d) and followed longitudinally for recurrence of vascularents.

.5.2. Lipid-Lowering Agenthere are no randomized trials to support specific lipid-wering drug therapy for a patient with stroke caused byheroembolism. However, 2 randomized studies of low-dosed higher-dose statin in patients with aortic and/or carotidaques showed significant regression in plaque seen on MR,
hich in 1 study was related to LDL cholesterol level but not ne
atin dosage (587) and, in the other study, was related to bothDL lowering and the statin dosage (588). It seems likely thatatin therapy decreases the risk of stroke. Mechanisms foris effect may involve pleiotropic effects of statins, includingaque regression, plaque stabilization, decreased inflamma-n, and inhibitory effects on the coagulation cascade at

fferent levels.No randomized trial on the use of statins in patients withvere aortic plaque has been published. However, in anservational study of 519 patients with severe aortic plaqueTEE (540), statin use was associated with an RR reduction

r ischemic stroke of 59%. Statins have reduced bothimary and secondary stroke in a variety of patient popula-ns (589,590). Hence, a majority of patients with stroke and

IA with identified aortic plaque already have other strongerdications for statin therapy (409). Recommendations foratins are noted for other manifestations of atheroscleroticseases (409) (see Section 9.2.1.2). Clinical trials are neededdetermine the effects of statins in patients with severe

rtic atheroma and risk of atheroembolism.

.5.3. Surgical and Interventional Approachesortic arch endarterectomy has been attempted for patientsith thromboembolism originating from aortic arch ather-

a. Although successful in a handful of case reports, thisocedure resulted in a relatively high rate (34.9% withdarterectomy versus 11.6% without endarterectomy) ofrioperative stroke and mortality when it was performed toit stroke during cardiac surgical procedures requiring

rdiopulmonary bypass (coronary bypass surgery and valvergery) (545). Covered stents offer the potential advantage ofielding severely diseased aortic segments to prevent furtherbolization. However, periprocedural embolization may

cur during diagnosis or interventional endovascular manip-ations. There is insufficient evidence to recommend pro-ylactic endarterectomy or aortic arch stenting for purposesstroke prevention.

2. Porcelain Aorta

ascular calcification occurs in the media and represents antral component of atherogenesis, typically signaling long-anding inflammation. The amount of calcification directlysociates with the extent of atherosclerosis, and the presence

aortic calcium predicts the presence of coronary heartsease (443).With severe atherosclerosis of the aorta, calcification maysevere and diffuse, causing an eggshell appearance seen onest x-ray or CT (444). Also termed “porcelain aorta,” thisding is usually noted during operation for coronary heart orlvular heart disease at the time of surgery. The calcificationterferes significantly with cannulation of the aorta, cross-amping, and placement of coronary bypass grafts, increas-g the risk of stroke and distal embolism significantlyigure 36).In these patients, direct manipulation of the aorta maysult in an unrepairable aortic injury and/or distal emboliza-n. Surgeons have used several techniques to reduce adverse
urologic events in these patients: internal aortic balloon

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clusion (as apposed to aortic cross-clamping), a “no-touch”chnique to avoid the ascending aorta, alternate locations fornnulation or coronary bypass graft anastomoses, replace-ent of the ascending aorta, and intra-aortic filtration ofherosclerotic debris (591–599).

3. Tumors of the Thoracic Aorta

eoplasms of the thoracic aorta are usually secondary andlated to contiguous spread of adjacent primary malignan-es, particularly lung and adjacent primary malignancies, orbsequent metastases, particularly lung and esophagus00–603).Primary neoplasms of the thoracic aorta are rare. A reviewthe literature between 1873 and 2002 collated a total of 53

oracic and 10 thoracoabdominal tumors with most lesionsotruding into the aortic lumen (604) (Table 16).Metastatic disease is often demonstrated at the time ofagnosis of primary aortic neoplasms, so that constitutionalmptoms of malaise, fatigue, weight loss, and nausea may bee presenting complaints. Other presentations can includestal arterial embolization (with histopathologic examinationowing neoplasm or identified by imaging techniques duringsearch for an embolic source) (604–606). AoD mayiginate in the area of the neoplasm or the aortic occlusion07). Resection and reconstruction of the segment of aortantaining the neoplasm have been described, but becauseost patients present with metastatic disease, overall prog-
sis is poor (608).
4. Perioperative Care for Openurgical and Endovascular Thoracicortic Repairs

4.1. Recommendations forreoperative EvaluationASS I

In preparation for surgery, imaging studies adequate to estab-lish the extent of disease and the potential limits of theplanned procedure are recommended. (Level of Evidence: C)Patients with thoracic aortic disease requiring a surgical orcatheter-based intervention who have symptoms or other find-ings of myocardial ischemia should undergo additional studiesto determine the presence of significant coronary artery dis-ease. (Level of Evidence: C)Patients with unstable coronary syndromes and significantcoronary artery disease should undergo revascularization priorto or at the time of thoracic aortic surgery or endovascularintervention with percutaneous coronary intervention or con-comitant coronary artery bypass graft surgery. (Level of Evi-dence: C)

ASS IIa

Additional testing is reasonable to quantitate the patient’scomorbid states and develop a risk profile. These may includepulmonary function tests, cardiac catheterization, aortogra-

Figure 36. Porcelain aorta. Top left and right, PA andlateral chest x-ray show an anterior mediastinal masswith curvilinear calcifications most likely representingthe wall of an ascending aortic aneurysm. Bottom left,CT scan slice at the level of the right pulmonary arteryconfirms a 10-cm aneurysm of the ascending aortawith dense mural calcifications. Bottom right, A maxi-mum intensity projection in the oblique sagittal planebetter demonstrates the fusiform aneurysm beginningat the sinotubular ridge and extending into the aorticarch. Dense mural calcification extends into the proxi-mal descending aorta. CT indicates computed tomo-graphic imaging; and PA, posteroanterior.

phy, 24-hour Holter monitoring, noninvasive carotid artery

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screening, brain imaging, echocardiography, and neurocogni-tive testing. (Level of Evidence: C)For patients who are to undergo surgery for ascending or archaortic disease, and who have clinically stable, but significant(flow limiting), coronary artery disease, it is reasonable toperform concomitant coronary artery bypass graft surgery.(Level of Evidence: C)

ASS IIb

For patients who are to undergo surgery or endovascularintervention for descending thoracic aortic disease, and whohave clinically stable, but significant (flow limiting), coronaryartery disease, the benefits of coronary revascularization arenot well established. (609–611) (Level of Evidence: B)

eoperative evaluation usually includes imaging studiescessary to establish the extent of disease, the limits of theanned procedure, and the clinical risks attendant to theocedure. When the writing committee was polled regardinge extent of usual preoperative laboratory testing, a variety

approaches emerged. In some centers, extensive testingcludes pulmonary function tests (particularly for smokersd those with Marfan syndrome), Holter monitoring, androtid duplex scans. In some centers, brain imaging andurocognitive testing are performed in patients with aorticch disease for whom arch repair or replacement requiring ariod of deep hypothermic circulatory arrest or low pumpw is planned (463). Other centers obtain fewer preopera-e tests and individualize such testing as cardiac catheter-

ation, 24-hour Holter monitoring, brain imaging, and neu-cognitive studies for patients to establish baseline states andsk.In many centers, where the diagnosis of acute AoD is eitherade or highly suspected, patients are immediately taken torgery, and TEE is performed in the operating room to eithertablish or confirm the diagnosis. Most of the writing

ble 16. Neoplasms of the Thoracic Aorta (Collectiveview Incidence)

stologyThoracic Aorta

(N�53)Thoracoabdominal

Aorta (N�10)

rcoma 15 1

alignant fibrous histiocytoma 11 1

giosarcoma 5 0

iomyosarcoma 6 2

rosarcoma 4 0

yxoma 3 1

romyxosarcoma 1 1

mangiopericytoma 2 0

mangioendothelioma 2 0

alignant endothelioma 2 0

rtic intimal sarcoma 0 2

yxosarcoma 0 1

dotheliosarcoma 1 0

romyxoma 0 1

roxanthosarcoma 1 0

Adapted from Oldenburg et al (604).

mmittee believes that the delay to obtain coronary angiog- di

phy was potentially dangerous unless patients had a historycoronary artery bypass graft surgery or a high likelihood ofexisting CAD.Most patients undergoing elective aortic root and ascend-g aortic surgery can be admitted the day of surgery.owever, some of the writing committee members routinelymit patients the day before surgery primarily for fluiddration (using 5% dextrose/0.5 normal saline with addi-nal potassium and sodium bicarbonate at 100 to 120 mL/h),rticularly those who are to have extensive open surgery forch, descending thoracic, or thoracoabdominal aortic dis-se. Preoperative use of acetylcysteine (600 mg by mouth atght and in the morning, or 500 mg in 500 mL of normalline solution over 3 hours before CT or surgery) has alsoen described (612,613). However, the effectiveness of these

rategies has not been tested in a clinical trial. Placement oforacic epidural catheters for postoperative analgesia ormbar spinal drains for CSF drainage is performed on they prior to surgery in some centers. Despite lack of evi-nce, there is concern that neuraxial hemorrhage is moreely if blood returns through the placement needle on they of surgery.

.1.1. Preoperative Risk AssessmentI, low cardiac output, respiratory failure, renal failure, androke are the principal causes of mortality and morbidityter operations on the thoracic aorta, and preoperativesessment of these organ systems prior to elective operationessential (381,441,614–617), especially in patients with astory of MI, angina pectoris, or symptoms of heart failured those older than 40 years. Patients with valvular heartsease are evaluated with echocardiography and cardiactheterization.Elderly patients with thoracic aortic disease are likely tove coexisting CAD. The benefits of prior or concomitantronary revascularization are controversial. Several studiesggested that prior coronary bypass graft surgery was ofnefit to patients undergoing major vascular surgery toduce cardiovascular mortality (618–622).More recent clinical trials comparing outcomes of patients

ith stable CAD treated with optimal medical therapy versusvascularization have not shown any significant reductions

cardiovascular events associated with revascularizationrategies (609–611). Major thoracic aortic surgery falls intoe highest-risk group for cardiac morbidity and mortality23), prompting some writing committee members to ag-essively screen for and treat coexisting CAD, but thefectiveness of such a strategy in patients with stable CADs not been clearly established. For patients with unstable

AD, left main stenosis, or 3-vessel disease, revasculariza-n is generally warranted prior to or concomitant with the

oracic aortic procedure. The use of drug-eluting stents forngle- or double-vessel disease may be problematic becausequired antiplatelet therapies may significantly raise the riskbleeding with the thoracic aortic procedure and withhold-

g antiplatelet therapies clearly increases the risk of stentrombosis.History of smoking and presence of chronic pulmonary

sease are important predictors of postoperative respiratory

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mplications and are frequently present in patients withoracic aortic disease (624). Pulmonary function tests andterial blood-gas analyses help to risk-stratify patients withronic pulmonary disease. If reversible restrictive disease orcessive sputum production is present, antibiotics and bron-odilators should be administered. Cessation of smoking isvisable.Preoperative renal dysfunction is the most important pre-

ctor of acute renal failure after operations on the thoracicrta (617,625,626). Preoperative hydration and avoidance ofpotension, low cardiac output, and hypovolemia in therioperative period may reduce the prevalence of thismplication.To minimize the risk of stroke or reversible ischemicurologic deficits and to determine the potential magnituderisk, duplex imaging of the carotid arteries and angiogra-y of the brachiocephalic arteries is often performed preop-atively particularly in patients with a history of stroke, TIA,other risk factors for cerebrovascular disease (627). How-er, the efficacy of treatment of significant carotid stenosisior to endovascular or open surgery for thoracic aorticsease has not been evaluated in a randomized clinical trial.Although older age is a risk factor for increased early andte death after operations on the thoracic aorta82,441,617,625,627–630), operations can be carried outccessfully with satisfactory outcomes in appropriately se-cted older patients. Emergency operation for aortic ruptureacute dissection is associated with a higher risk of early

ath after operation compared with elective operation (47).

4.2. Recommendations for Choice ofnesthetic and Monitoring TechniquesASS I

The choice of anesthetic techniques and agents and patientmonitoring techniques should be tailored to individual patientneeds to facilitate surgical and perfusion techniques and themonitoring of hemodynamics and organ function. (Level ofEvidence: C)

ASS IIa

Transesophageal echocardiography is reasonable in all opensurgical repairs of the thoracic aorta, unless there are specificcontraindications to its use. Transesophageal echocardiogra-phy is reasonable in endovascular thoracic aortic proceduresfor monitoring, procedural guidance, and/or endovasculargraft leak detection. (631–633) (Level of Evidence: B)Motor or somatosensory evoked potential monitoring can beuseful when the data will help to guide therapy. It is reasonableto base the decision to use neurophysiologic monitoring onindividual patient needs, institutional resources, the urgencyof the procedure, and the surgical and perfusion techniques tobe employed in the open or endovascular thoracic aortic repair.(634,635) (Level of Evidence: B)

ASS III

Regional anesthetic techniques are not recommended in pa-tients at risk of neuraxial hematoma formation due to thien-
opyridine antiplatelet therapy, low-molecular-weight heparins, tio
or clinically significant anticoagulation. (636) (Level of Evi-dence: C)Routinely changing double-lumen endotracheal (endobron-chial) tubes to single-lumen tubes at the end of surgicalprocedures complicated by significant upper airway edema orhemorrhage is not recommended. (Level of Evidence: C)

hoice of anesthetic technique is dependent on the plannedrgical interventions and the patient’s comorbid conditions.r placement of endovascular aortic stent grafts, differentesthetic (local, regional, general) techniques have beenscribed, although adequate comparative studies are lacking37–643). Percutaneous placement of catheters with a lim-d incision may be tolerated with local anesthesia anddation. Extensive inguinal dissection or the construction offemoro–femoral bypass may favor either regional or generalesthesia. If surgical dissection is extended into the retro-ritoneum, a higher level of regional anesthesia or generalesthesia is required. If the patient is undergoing localesthesia or regional anesthesia, adequate intravenous seda-n is necessary because of agitation secondary to restless-ss and pain from lying in one position for a prolongedriod of time.Retrospective studies indicate that patients having localrsus regional or general anesthesia for endovascular stentafts tend to have lower use of vasoactive agents and lowerid requirements, shorter intensive care and hospital stays42), earlier ambulation and gastrointestinal function (637),d lower incidence of respiratory and renal complications43). In a large multicenter retrospective study of 5557tients undergoing endovascular aortic repairs (644), 69%ceived general anesthesia, 25% received regional anesthe-a, and 6% received local anesthesia. The incidence ofrdiac complications were significantly lower in both thecal or regional anesthesia group compared with generalesthesia (1.0% versus 2.9% versus 3.7%), and the inci-nce of sepsis was significantly lower in the regionalesthesia group compared with general anesthesia (0.2%rsus 1.0%). Selection bias and complexity of disease likely

fect these results.

.2.1. Temperature Monitoringt most centers, temperature is monitored in at least 2cations that estimate the brain/core (e.g., blood, esophageal,mpanic membrane, nasopharynx) temperature and the vis-ral (e.g., bladder or rectal) temperature (645).

.2.2. Hemodynamic Monitoringvasive arterial pressure monitoring is required in 1 or moretes depending on the surgical plan for cannulation andrfusion, particularly for thoracoabdominal aortic repairs.rterial pressure is universally monitored proximal to aorticoss-clamping sites, but there is institutional variability ine monitoring of distal arterial (aortic) pressure, even whenstal aortic perfusion is performed.Central venous cannulation allows measurement of cardiac

ling pressures, providing a route for vasoactive drug and fluidministration. Femoral venous catheterization is discouragedcurrent central line–associated bloodstream infection preven-
n guidelines, but the literature does not address the subject in

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oracoabdominal surgery (646,647). Nevertheless, many expe-enced centers use short-term catheterization of the femoral veinr volume management during surgery.Pulmonary artery catheterization is performed routinely in

any institutions for thoracic aortic surgery. The literaturees not specifically address the subject of thoracic aorticrgery, but the general perioperative literature does notpport the use of pulmonary artery catheterization (631).

.2.3. Transesophageal EchocardiographyEE is safe (648) and is used to confirm the preoperativeagnoses and detect pericardial or pleural effusions, aorticgurgitation, the extent of dissection, the location of intimalars, the size and integrity of aneurysms, and the presence ofpropriate flow in the true lumen upon commencement ofrdiopulmonary bypass. Current American Society of Anes-esiologists and the Society of Cardiovascular Anesthesiol-ists guidelines for TEE include the following (631):Category I Indications (supported by the strongest evi-nce or expert opinion):

Preoperative use in unstable patients with suspected tho-racic aortic aneurysms, dissection, or disruptions that needto be evaluated quickly.Intraoperative assessment of aortic valve function in repairof AoDs with possible aortic valve involvement.

Category II Indications (supported by weaker evidence orpert opinion):

Preoperative assessment of patients with suspected tho-racic AoDs, aneurysms, or disruption.Intraoperative use during repair of thoracic AoDs withoutsuspected aortic valve involvement.

.2.4. Transesophageal Echocardiography forndovascular Repairs of the Descending Thoracic AortaEE can provide views of the aorta and location of guide-ires and endografts prior to deployment in relation to thermal and diseased thoracic aorta (649,650). TEE hasstinct advantages over angiography by providing exactssel and lesion sizing and localization and can also be usedestimate endograft size and location. Although not imagedall patients, large intercostal arteries have been imaged,

us avoiding inadvertent obstruction by the aortic stent graft.fter stent graft placement, the presence or absence ofdoleaks can be determined by TEE with a high degree ofnsitivity and specificity particularly compared with contrastgiography (632,633,650). Finally, because most of thesetients have severe concomitant cardiac disease, periopera-e TEE allows dynamic assessments of cardiac function.Intravascular ultrasound is used for endovascular proce-res to visualize precise localization of branch arteries andr measurement of aortic and branch artery sizes. Plaques,ars, and saccular aneurysms can also be very accuratelymonstrated.

4.3. Airway Management for Descendingoracic Aortic Repairsdouble-lumen endotracheal tube or various types of endo-
onchial blockers facilitate surgical exposure (651). For se
tensive surgery of the left thorax, single-lung ventilationovides better surgical exposure, reduces the need forlmonary retraction, may decrease the severity of iatrogeniclmonary contusion, and protects the right lung from con-

mination by blood and secretions. A large descendingoracic aortic aneurysm may compress or distort the leftain bronchus such that left-sided endobronchial tubes mustused with caution. If a right-sided double-lumen endotra-

eal tube is placed, endoscopic confirmation of tube positiono ensure right upper lobe ventilation) is necessary. Forcefuldobronchial tube placement has been associated with tho-cic aortic aneurysm rupture. Therefore, using a differentbe or lung isolation method may be required when severerway distortion is encountered.At the end of surgery, some centers have advocatedanging a double-lumen endotracheal tube to a single-lumenbe to facilitate pulmonary toilet and to avoid the complica-ns associated with tube malposition in the intensive careit. The decision to change the double-lumen endotrachealbe to a single-lumen tube should be made after carefullyaluating the extent of airway edema, as these procedurese associated with significant facial and laryngeal edema.dvanced airway management devices, such as tube ex-anges and video laryngoscopy, may be of benefit; however,ere is no literature addressing this subject.

4.4. Recommendation for Transfusionanagement and Anticoagulation in Thoracicortic SurgeryASS IIa

An algorithmic approach to transfusion, antifibrinolytic, andanticoagulation management is reasonable to use in both openand endovascular thoracic aortic repairs during the periopera-tive period. Institutional variations in coagulation testing ca-pability and availability of transfusion products and otherprothrombotic and antithrombotic agents are important con-siderations in defining such an approach. (652) (Level ofEvidence: C)

horacic aortic surgery is associated with hemorrhage fromveral interrelated causes, including extensive surgical dis-ction, arterial and venous bleeding, hypothermia, cardio-lmonary bypass, fibrinolysis, dilution or consumption ofagulation factors, thrombocytopenia, poor platelet function,parin and other anticoagulants, preoperative antithromboticerapy, and other congenital and acquired coagulopathies.he extensive blood product and fluid requirements of openoracic aortic surgical repairs may exceed the total bloodlume of the patient in the most complicated cases. Clinicalactice guidelines for perioperative blood transfusion andood conservation in cardiac surgery have been published bye STS and the SCA (652).These guidelines do not specifically address open ordovascular descending thoracic aortic repairs, but theriting committee supports treatment strategies provided byese guidelines.Coagulopathies in open descending thoracic aortic andoracoabdominal repairs mirror many of the abnormalities
en in cardiac and thoracic aortic procedures requiring

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rdiopulmonary bypass. Illig and colleagues (653) reportedgnificantly decreased euglobulin clot lysis times, elevatedsue plasminogen activator levels, elevated tissue plasmin-en activator–to–plasminogen activator inhibitor-1 ratios,d reduced alpha 2-antiplasmin levels within 20 minutester supraceliac cross-clamping, compared with infrarenalclusion, consistent with a primary fibrinolytic state. Vis-ral ischemia may be the condition that initiates fibrinolysis.uring supraceliac occlusion, fibrinolysis was attenuatedhen superior mesenteric artery perfusion was maintaineda a shunt (654). Peripheral ischemia may also result inrinolysis. Within 30 minutes of the onset of acute periph-

al ischemia (with infrarenal aortic occlusion) fibrinolytictivity increased, as demonstrated by an increase in tissue-pe plasminogen activity and a decrease in plasminogentivator inhibitor activity. This increase in tissue-type plas-inogen activity resulted from release of stores from isch-ic vascular tissues (655). Endotoxemia during aortic oc-

usion may also be associated with fibrinolysis (656).To counteract fibrinolysis, the use of lysine analogssilon-aminocaproic acid and tranexamic acid has beenported in cardiac surgery. The epsilon-aminocaproic acidading or bolus dose ranged from 75 to 150 mg/kg, withditional dosing from 12.5 to 30 mg/kg/h infused overrying time periods. For tranexamic acid, loading or bolusse, ranged from 2.5 to 100 mg/kg, with additional dosing

om 0.25 to 4.0 mg/kg/h delivered over 1 to 12 hours (657).a study of 21 adult cardiac surgical patients, the tranexamicid dosing regimen of 10 mg/kg initial dose, followed by anfusion of 1 mg/kg/h resulted in adequate plasma concen-ations defined by in vitro studies to prevent fibrinolysis,ith relatively stable drug levels throughout cardiopulmonarypass (658). Antifibrinolytic therapy for thoracoabdominalrtic surgery with distal perfusion was not associated withcreased bleeding or transfusion in a retrospective cohort ofpatients (659). The strong evidence derived from other

rdiac surgical studies has led to very common use oftifibrinolytic therapy in thoracic aortic surgery, despite thesence of specific evidence in this surgical subset.

4.5. Organ Protection

.5.1. Recommendations for Brain Protection Duringscending Aortic and Transverse Aortic Arch SurgeryASS I

A brain protection strategy to prevent stroke and preservecognitive function should be a key element of the surgical,anesthetic, and perfusion techniques used to accomplishrepairs of the ascending aorta and transverse aortic arch.(660–666) (Level of Evidence: B)

ASS IIa

Deep hypothermic circulatory arrest, selective antegrade brainperfusion, and retrograde brain perfusion are techniques thatalone or in combination are reasonable to minimize brain injuryduring surgical repairs of the ascending aorta and transverseaortic arch. Institutional experience is an important factor inselecting these techniques. (211,615,667–688) (Level of
Evidence: B) m
ASS III

Perioperative brain hyperthermia is not recommended in re-pairs of the ascending aortic and transverse aortic arch as it isprobably injurious to the brain. (689–691) (Level of Evi-dence: B)

brain protection strategy is an essential component of theerative technique for open surgical repairs of the ascendingrta and/or the aortic arch. Moderate or profound hypother-ia with periods of circulatory arrest and/or selective ante-ade brain perfusion and/or retrograde brain perfusion aree common strategies for achieving brain protection. Theperience and outcomes of the operating surgeon and thestitution are important considerations in selecting a brainotection strategy.Achieving brain hypothermia is nearly universally per-rmed using extracorporeal circulation, with temperaturesnging from 12° to 30°C. Retrograde (via jugular vein) brainrfusion is usually performed at a perfusion pressure of 20 tomm Hg at a mildly or profoundly hypothermic tempera-

re. Antegrade brain perfusion is usually performed at arfusion pressure of 50 to 80 mm Hg and may be instituted

direct cannulation of the brachiocephalic arteries, side-aft anastomosis to the axillary artery, or direct cannulationa portion of graft material that was anastomosed to the

achiocephalic arteries during a period of hypothermicrculatory arrest. The rewarming of a patient followingmpletion of the repair of the thoracic aorta is usuallyrformed at a measured rate so as not to induce brainperthermia.The reviewed literature describes an evolution of brainotection techniques over the past 2 to 3 decades. Deeppothermic circulatory arrest emerged as the first technique,t as a sole method of brain protection, it was limited bycreasing rates of neurologic morbidity, other adverse out-mes, and mortality as the period of arrest exceeded 25 to 45inutes (671,692). Deep hypothermic arrest without perfu-on adjuncts has been successful, especially when arresttervals are less than 40 minutes (693). Subsequently,rious combinations of retrograde brain perfusion and se-ctive antegrade brain perfusion were developed to extende “safe period” of interruption of full extracorporeal circu-tion. Monitoring of brain function and metabolic suppres-on by electroencephalography, evoked potentials, bispectraldex, noninvasive cerebral oximetry, and jugular bulb oxy-moglobin saturation are additional means used to guide theset of extracorporeal circulation interruption for repairinge distal ascending aorta and/or aortic arch. Some centers userbiturates, calcium channel blockers, or steroids for addedotection, but no prospective randomized trials have beenrformed to test the efficacy of pharmacological agents11,459).There is controversy regarding the ability of retrogradeain perfusion to support brain metabolic function and toprove neurologic outcomes, including transient postopera-

ve neurologic dysfunction, stroke rates, and mortality61,671,673–675,694–699). However, this technique can
aintain brain hypothermia (700) and has been associated

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ith improved outcomes in the centers where it is used as aimary neuroprotection strategy.Selective antegrade brain perfusion may be provided byrect cannulation of 1 or more of the brachiocephalicteries, which permits brain perfusion with minimalriods of interruption. If unilateral cannulation is per-rmed, success may depend on patency of the circle ofillis. Alternatively, unilateral direct or side-graft cannu-tion of the (usually right) axillary artery permits extra-rporeal circulation and cooling without manipulation ofe diseased thoracic aorta. This same cannula can then beed for delivering antegrade brain perfusion immediatelyter the section of aorta from which the brachiocephalicteries originate is sutured to the graft or immediatelyter the brachiocephalic vessels are individually anasto-osed to a trifurcated graft. The time required to completeese maneuvers requires a relatively shorter period ofpothermic circulatory arrest compared with competeconstruction of the aortic arch (681,683,701–704). Bilat-al brachiocephalic artery cannulation has also beenported (705). The literature is insufficient to determinehether unilateral or bilateral perfusion or complete avoid-ce of circulatory arrest is associated with improvedtcomes. A retrospective analysis by Svensson et al (449)ggested that axillary artery perfusion via a prostheticde-graft was associated with improved outcomes com-red with femoral arterial cannulation.Finally, direct cannulation of the aortic replacement graft

ay be used to institute antegrade brain perfusion followingperiod of circulatory arrest. The variability of techniquesong surgical centers makes direct comparison difficult;wever, most studies with some type of antegrade arterialain perfusion report outcomes that are comparable to ortter than those using hypothermic circulatory arrest aloneretrograde brain perfusion (660,662,666,685,686,706,707).rthermore, selective antegrade brain perfusion may reduce

e period of brain ischemia and permit less profoundpothermia, which may be associated with good clinicaltcomes (676,687,708–712).Our ability to create evidence-based guidelines from theerature is particularly difficult in the case of brain protec-n. Changes in surgical technique, perfusion technology,esthetic and intensive care management, coagulation man-ement, prosthetic graft materials, and the experience of theporting centers and lack of randomized clinical trials arenfounding factors.

.5.2. Recommendations for Spinal Cord Protectionuring Descending Aortic Open Surgical andndovascular RepairsASS I

Cerebrospinal fluid drainage is recommended as a spinal cordprotective strategy in open and endovascular thoracic aorticrepair for patients at high risk of spinal cord ischemic injury.(522,523,713) (Level of Evidence: B)

ASS IIa

Spinal cord perfusion pressure optimization using techniques,
such as proximal aortic pressure maintenance and distal aortic re
perfusion, is reasonable as an integral part of the surgical,anesthetic, and perfusion strategy in open and endovascularthoracic aortic repair patients at high risk of spinal cordischemic injury. Institutional experience is an important factorin selecting these techniques. (380,382,714,715) (Level ofEvidence: B)Moderate systemic hypothermia is reasonable for protection ofthe spinal cord during open repairs of the descending thoracicaorta. (525) (Level of Evidence: B)

ASS IIb

Adjunctive techniques to increase the tolerance of the spinalcord to impaired perfusion may be considered during open andendovascular thoracic aortic repair for patients at high risk ofspinal cord injury. These include distal perfusion, epiduralirrigation with hypothermic solutions, high-dose systemic glu-cocorticoids, osmotic diuresis with mannitol, intrathecal pa-paverine, and cellular metabolic suppression with anestheticagents. (520,715–717) (Level of Evidence: B)Neurophysiological monitoring of the spinal cord (somatosen-sory evoked potentials or motor evoked potentials) may beconsidered as a strategy to detect spinal cord ischemia and toguide reimplantation of intercostal arteries and/or hemody-namic optimization to prevent or treat spinal cord ischemia.(483,634,718,719) (Level of Evidence: B)

raparesis and paraplegia are perhaps the most fearedmplications following repair of the descending thoracicrta. Although rates as high as 23% have previously beenported, the current incidence is probably somewhere around

to 6% (483,521,634,718,720). In any specific patient,wever, the likelihood of neurological complications de-nds highly on individual anatomy, on whether the aorta isssected or aneurysmal, and on whether the pathology isute, chronic, or both.Risk factors for perioperative spinal cord injury includeergency surgery, dissection, extensive disease, prolonged

rtic cross-clamp time, aortic rupture, level of aortic cross-amp, patient age, prior abdominal aortic surgery, and, inrticular, hypogastric artery exclusion (143,144,721), asell as a history of renal dysfunction. The risk of paraplegiaparaparesis is minimal if the aortic cross-clamp time is less

an 15 minutes (722). Svensson et al (381) reported a 20%sk of neurological injury if aortic cross-clamp time waseater than 60 minutes and less than 10% if aortic cross-amp time was less than 30 minutes. Although paraplegia orraparesis has been reported with aortic cross-clamp timesless than 20 minutes (723), others have concluded that

rtic cross-clamp times greater than 40 minutes did notsult in increases in adverse spinal cord outcome, if distalrfusion was used (382). One study of patients undergoingen or endovascular repair of descending thoracic or thora-abdominal aortic aneurysms demonstrated a higher risk ofinal cord injury related to the extent of aorta treated but nofference between the operative approaches (724).Another option for spinal cord protection is deep hypother-

ic circulatory arrest (630). This has been a useful techniquer complex descending thoracic or thoracoabdominal aortic
pairs performed via a left thoracotomy approach.

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.5.2.1. MONITORING OF SPINAL CORD FUNCTION IN

ESCENDING THORACIC AORTIC REPAIRS

general, the comparatively low current incidence of neu-logic complications is attributable to the routine use ofultimodal neurophysiologic monitoring such as somatosen-ry evoked potentials (SSEP) and motor evoked potentialsEP) in conjunction with neuroprotective strategies noted

ter (526). Although these neuroprotective strategies con-ue to evolve and may differ slightly in their implementation

om center to center, monitoring of evoked potentials duringese cases has become common because it provides thergeon and anesthesiologist the opportunity to promptlytervene if alterations in monitored potentials indicate neu-logic compromise is occurring (524,526,719,725,726).

SSEPs are cerbral cortical electrical potentials recordedith scalp electrodes during electrical stimulation of thesterior tibial or peroneal nerves of the lower extremities,nducted via the lateral and posterior columns of the spinalrd (727). Because SSEP monitoring is less sensitive toesthetic drugs and paralytic agents may be used, its use isss complex than MEP monitoring. SSEP monitoring is

ited because it is only dependent on the integrity of theteral and posterior columns. The anterior motor column isore likely to be affected by ischemic injury during aorticconstruction. It is thus possible to sustain an isolatedrioperative anterior column injury without changes inEPs. In 1 study, SSEP monitoring influenced the surgical

rategy in 17 of 63 patients (27%) undergoing descendingrtic reconstruction (719). Corrective interventions includedrtial cardiopulmonary bypass initiated in 1 patient with

aumatic aortic rupture; reimplantation of critical intercostal,mbar, or sacral arteries in 11 patients; suture closure ofofusely back-bleeding intercoastal arteries in 1 patient;stening the proximal suture line in 1 patient; distal clamppositioning to a more proximal position in 1 patient; andoximal clamp repositioning in 2 patients with left carotidchemia. The authors reported no cases of unexplained SSEPnormalities. New immediate paraplegia was observed in 1tient with sustained SSEP absence, and 2 patients presentedith delayed paraplegia despite normal inoperative SSEP.

In contrast, MEPs are performed by stimulating the motorrtex (by either high-voltage short-duration electrical stim-us or magnetic induction) (728) and recording at the level

the spinal cord, peripheral nerves, or muscles24,526,725–727). Neurogenic MEPs are responses re-rded at the peripheral nerves, whereas myogenic MEPs arerge biphasic responses recorded over the muscle belly.ecause the amplitude of the response is proportional to thember of motor neurons being stimulated, these evokedtentials are very sensitive to neuromuscular blocking andesthetic agents (729–738).

Monitoring of SSEPs alone has not been demonstrated toprove outcomes in patients undergoing TAA repair. SSEP

onitoring is associated with delayed ischemia detectionmpared with transcranial MEPs (739), as well as high ratesboth false-negative and false-positive results (635,739).

yon et al (740) reported a significantly higher voltage
reshold for the generation of a 50 microV amplitude signal co
the end of the procedure compared with the beginning ofe procedure (“anesthetic fade”). This increased voltagereshold was directly proportional to the length of anestheticposure. Recognition of this phenomenon is important tooid false-positive MEP interpretation.

To demonstrate the greater sensitivity of MEP than SSEPonitoring, Dong et al (634) reported their experience with

patients undergoing descending aortic reconstruction. Alltients were monitored with both MEPs and SSEPs. Sixteentients (29%) had MEP evidence of perioperative spinalrd ischemia compared with 4 patients (7%) with SSEPanges. These changes were reversed in 13 patients withther segmental artery reimplantation or optimization ofmodynamics. Although one of these 13 patients awokeith immediate paraplegia, the remaining 3 patients awokeraplegic. All 3 patients had normal perioperative SSEPs.

.5.2.2. MAINTENANCE OF SPINAL CORD ARTERIAL PRESSURE

oximal hypertension may increase the contribution of thertebral artery–derived blood flow to the spinal cord as wellcollateral flow. Other methods of maintaining arterial flowthe spinal cord include the aggressive reimplantation of

ajor intercostal arteries into the aortic graft (715). Griepp et(742) emphasized the importance of prompt ligation ofnimplanted intercostal arteries to avoid “steal” from bleed-g during periods of ischemia to this collateral bed (741).trathecal papaverine has also been described as a method ofducing spinal cord arterial dilation, and thus increasinginal cord blood flow.

The literature is unclear with respect to the benefits ofstal perfusion alone on spinal cord protection because thechnique is not used in isolation. Several studies support thencept that distal perfusion combined with CSF drainage isneficial (380,382,468,714). The minimum desirable distalterial pressure is 60 mm Hg to ensure adequate spinal cordood flow, whereas maximal proximal mean arterial pressureould be about 90 to 100 mm Hg (743).

.5.2.3. CEREBROSPINAL FLUID PRESSURE AND DRAINAGE

pplication of a cross-clamp to the proximal descending aortat only creates a major hemodynamic load on the heart but alsouses an acute elevation in CSF pressure (744). Surgicaltraction of the aortic arch may also produce significant in-eases in CSF pressures (745). When CSF pressure exceedsinal venous pressure, a “critical closing pressure” is achieved,d the veins collapse independent of inflow pressure. Theinal cord perfusion pressure is therefore the difference betweeninal arterial pressure and CSF pressure.

Coselli et al (522) randomized 145 patients undergoingoracoabdominal aortic repair with or without CSF drainage.ine patients (13.0%) in the control group developed para-egia or paraparesis. In contrast, only 2 patients in the CSFainage group (2.6%) had deficits develop (P�0.03). Notients with CSF drainage had immediate paraplegia. Aeta-analysis by Khan and Stansby (713) and the retrospec-e analysis by Safi and colleagues (523) also concluded that

SF drainage was advantageous in reducing the risk of spinal
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Possible complications of CSF drainage include headache,inal or epidural hematoma formation or inflammatoryaction, meningitis, and persistent CSF leaks. Subduralmatoma has been reported after thoracic aortic repair withinal fluid drainage (746). Introduction of blood into thebarachnoid space may result in vasospasm and decreases ininal blood flow (747). Decreases in CSF pressure maycur with phlebotomy, and aggressive use of hyperosmoticents and hyperventilation may be as effective as spinalainage in maintaining spinal cord perfusion pressure48,749). In a single-center report of 162 patients with CSFains, 6 patients (3.7%) had catheter-related complications:mporary abducens nerve palsy, 1 patient; retained catheteragments, 2 patients; retained catheter fragments and men-gitis, 1 patient; isolated meningitis, 1 patient; and spinaladache, 1 patient. There were no neuraxial hemorrhagicmplications in this series (750).

.5.2.4. HYPOTHERMIA

ild hypothermia may provide significant neuronal protec-n by mechanisms such as reducing excitatory neurotrans-

itter release, decreasing free oxygen radical production,creasing postischemic edema, and stabilizing central ner-us system blood flow (751,752). Hypothermia occurs viassive cooling in a cold operating room with a majorcision in addition to cooling blankets and unwarmed intra-nous fluids. If an extracorporeal circuit is used, a heatchanger permits warming or cooling of the body tempera-re. A temperature of 32°C is usually well tolerated bytients not undergoing full cardiopulmonary bypass. Mod-ate systemic hypothermia has been associated with im-oved outcomes following TAA surgery (525). Arrhythmias,ch as atrial fibrillation and even ventricular fibrillation, cancur if hypothermia is too severe.

Epidural infusion of cooled saline may be used to inducegional hypothermia. Although this technique was associ-ed with substantial increases in CSF pressure, a significantduction in postoperative neurologic deficits was noted20,716). A new, self-contained catheter for topical coolingthe spinal cord without infusion into the CSF or CSF

essure rise has been shown in the laboratory to providepical spinal cord hypothermia, while systemic normother-ia is maintained; clinical trials are being organized (753).

Postischemic hyperemia occurs in the spinal cord (754). Theagnitude of this hyperemia has been demonstrated to beoportional to the incidence of paraplegia. Possible mecha-sms for the increased neurologic injury associated with vas-lar hyperemia include edema formation with the developmenta compartment syndrome and subsequently decreased spinalrd perfusion and increased oxygen delivery that may result ineater free oxygen radical species generation.

.5.2.5. GLUCOCORTICOIDS AND MANNITOL

dministration of methylprednisolone (30 mg/kg) before andter aortic occlusion may result in better spinal cord protec-n (717). The mechanism of this protection is unclear, but it

ay be related to decreased spinal cord edema and improvedee oxygen radical scavenging. Similarly, mannitol (0.25 to
0 g/kg) has been used to modulate the extent of ischemic fa
inal cord injury. Mannitol is hypothesized to act in similarshion to methylprednisolone (715).

.5.3. Recommendations for Renal Protectionuring Descending Aortic Open Surgical andndovascular RepairsASS IIb

Preoperative hydration and intraoperative mannitol administra-tion may be reasonable strategies for preservation of renalfunction in open repairs of the descending aorta. (Level ofEvidence: C)During thoracoabdominal or descending aortic repairs withexposure of the renal arteries, renal protection by either coldcrystalloid or blood perfusion may be considered. (626,755,756) (Level of Evidence: B)

ASS III

Furosemide, mannitol, or dopamine should not be given solelyfor the purpose of renal protection in descending aortic repairs.(757,758) (Level of Evidence: B)

a cohort of 475 patients who underwent descendingoracic aortic repair, 25% developed acute postoperativenal failure, whereas 8% required hemodialysis (759). Riskctors that are associated with postoperative renal failureter descending thoracic aortic repair include age greateran 50 years, preexisting renal dysfunction, duration of renalchemia, administration of greater than 5 units or eithercked red cells or salvaged washed autologous blood,modynamic instability, and diffuse atherosclerosis26,759).There is controversy regarding the protective nature of

stal perfusion during aortic occlusion (626,759). Godet et al59) observed a decrease in the incidence of renal failureith the use of distal aortic perfusion. Others have observedcreases in renal failure with distal perfusion (626). Selectivenal artery perfusion during descending thoracic aortic repairay result in uninterrupted urine production throughout theocedure and may decrease the incidence of renal failurestoperatively (755).Pharmacological agents, including mannitol (760,761),rosemide (758), or dopamine (757,761–764), have not beenmonstrated to provide renal protection during descendingoracic aortic repair.

4.6. Complications of Open Surgicalpproachesyocardial infarction (1% to 5%): This is an uncommonmplication but it is associated with CAD or dissection ofe coronary artery ostia (228–234,250,441).Heart failure (1% to 5%): Myocardial protection difficul-s and ventricular distention from either aortic valve regur-tation or high right-sided pressures are often factors (765).Infections (1% to 5% superficial, less than 1% deep):traoperative contamination or inadequate or improperly

ed antibiotic coverage, obesity, immunosuppression, pul-onary disease, or suboptimal glucose control may be a
ctor (766).

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Stroke (2% to 8% permanent): As noted earlier, brainotection is important in preventing the complication ofroke. The causes are either embolic or ischemic. Patientsho on preoperative MR have evidence of ischemic changesd/or reduced neurocognitive function, who are elderly, orho have a history of stroke are at increased risk of arioperative stroke (76,99,139,211,449,453,466,767).Neurocognitive deficit: The exact incidence of deficits hast been studied much after circulatory arrest; however, in 1ospective randomized study, using 51 neurocognitive tests,2 to 3 weeks after surgery, 9% of patients had new deficits,d by 6 months, all new deficits had resolved. Patients witheoperative deficits were proved to have further deteriora-n; indeed, 38% of patients had preoperative deficits11,466).Reoperation for bleeding (1% to 6%): Reoperation foreeding is dependent on the extent of surgery, length ofrdiopulmonary bypass, reoperative status, underlying dis-der, and surgical technique. Obtaining hemostasis prior toming off cardiopulmonary bypass and correcting all bloodagulation defects are paramount (765,766).Respiratory failure (5% to 15%): Preoperative pulmonarynction testing helps to warn the surgeon about potentialstoperative respiratory problems. Sometimes operative

chnique can be adapted to lessen the risks—for example,ortening pump time and avoiding overtransfusion. Leftaphragmatic incisions and trauma may add to postoperativelmonary dysfunction. In patients with more than 7 kg ofcreased weight after surgery, delaying extubation untilcess fluid has been eliminated by diuresis is worthwhile.hite cell filtration and plasmapharesis on pump may beeful but have not been tested in randomized trials.Ventricular arrhythmias (1% to 5%): Ventricular tachycar-a or ventricular fibrillation was a common complicationter composite valve graft insertion a decade ago, withports of 19% to 21% risk. Some of these events were relatedundiagnosed myocardial ischemia from coronary button

attachment problems, which are usually apparent duringeration or shortly thereafter. Inadequate myocardial protec-n may also lead to both ventricular arrhythmias and lowrdiac output. The increasing use of amiodarone, optimiza-n of potassium and magnesium levels, and better methodsmyocardial protection including blood cardioplegia may

ve reduced this risk. Twenty-four–hour Holter monitoringudies may reveal underlying pathology, such as ischemicanges or prolonged QT interval, particularly in patientsith Marfan syndrome, that needs to be addressed.Paralysis: The most feared complication after these typesoperations is lower limb paralysis and neurogenic bladder.

his occurs in 2% to 4% of descending thoracic aortic repairsd in 3% to 10% of thoracoabdominal aortic repairs82,526,724,768–773). Most lesions predominantly involveotor function because the anterior motor nerve cells of theinal cord are most likely to be involved. The protectiveeasures were discussed earlier. Two thirds of patients withraparesis will recover, and about half with paraplegia willcover to the point of walking again. Prevention of postop-ative hypotension in the intensive care unit and continued
SF drainage for longer than 40 hours is deemed by most le
thors to be beneficial in reducing the incidence of parapa-sis (380,522,523,525,713).Hoarseness: The incidence of hoarseness is related to

hether the arch needs to be clamped and whether theoximal descending aorta at the left subclavian artery needsbe transected. Transsection at this level is recommended tooid damage to the esophagus and to prevent the formationlate aortoesophageal fistulae. This, however, may result inmage to the recurrent laryngeal nerve as it wraps around therta and ligamentum arteriosum, resulting in left vocal cordralysis. This can usually be improved by vocal cordjection with either gel or collagen.

4.7. Mortality Risk for Thoracicortic Surgeryxpected results for risk of death are summarized next.Composite valve graft (1% to 5%): With modern tech-ques, death after elective repair is unusual (76,98,99,448).dditional comorbid states and the need for emergencyeration are associated with increased risk (765).Separate aortic valve replacement with ascending aortapair (1% to 5%) (448,628): Comorbid conditions such asvanced age and concomitant coronary bypass graft surgery,well as emergency operation, are associated with increased

sk (76,99,448,765,774,775).Valve-sparing aortic root reconstruction (less than 1% to5%): These patients are mostly young and otherwisealthy, and thus excellent results are expected comparedith other aortic operations. Indeed, in 1 series of over 200odified David reimplantations, there were no operativeaths (99). Late 10-year freedom from reoperation is betteran 92% for reimplantation procedures but lower for rootmodeling procedures (76,95,98,99,448,776).Bicuspid aortic valve and ascending aorta repair (1.5%):a large series of over 2000 patients with bicuspid aortic

lve surgery, the operative risk of death (1.5%) for patientsho had both a bicuspid valve procedure and ascendingrtic repair (n�200) was no different than the risk for thoseho underwent only a bicuspid valve procedure. For patientsith Marfan syndrome or connective tissue disorders orcuspid aortic valves, the long-term prognosis is excellentd reaches an average survival of 70 years (76,99). Late-year risk of reoperation is not as low as for tricuspid aorticlve repair but still is only 9% for bicuspid aortic valvepairs.Acute AoD (3.5% to 10% in experienced centers, butgher overall): The risk of death after surgical repair of acuteoD is strongly influenced by associated stroke, mesentericchemia, renal failure, and myocardial ischemia37,375,765,777).Total arch replacement: A 2% to 6% risk of death and a

to 7% risk of stroke have been reported for thesetensive and high-risk procedures. Emergency operationortality and stroke rates are higher (15% and 14%, respec-ely) (778). Careful brain and myocardial protection, cor-

ction of coagulopathies, and improved operative tech-ques, including the use of elephant trunk procedure, have
d to improved outcomes (680,779).

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Reoperations: The risk factors for reoperations are comor-d disease and extent of surgery with results varying be-een 2% to 6% for the risk of death (464).Descending aortic replacement: The risk of death withrrent techniques is 2% to 5% and the risk of paralysis is lessan 3% for elective surgical repairs (382).Thoracoabdominal repairs: The risk of death is stronglyfluenced by the urgency of surgery, comorbid disease, andtent of repair. Thus, Crawford Type I thoracoabdominalpairs have a risk of death of approximately 5%, but this isubled to approximately 10% for Type II repairs. The resultspicted earlier represent the work performed in high-volumenters and may not reflect results of all institutions at whichch surgery is performed (381,525,768,773,780,781).The late risk of death after aortic repair is stronglyfluenced by age and comorbid disease. Furthermore, aorticherosclerosis disease is a marker for more extensive ath-osclerosis. For patients undergoing degenerative and AoDpairs, usually at an average age of the lower 70s, 5-yearrvival rates of only 60% have been reported for ascendingpairs, arch repairs, descending repairs, thoracoabdominalpairs, and infrarenal repairs (3,381). Thus, it is initiallyportant to identify and to treat all comorbid disease,rticularly CAD, which is the most common cause of lateaths in this population (381). In patients undergoing elec-e root-sparing procedures or bicuspid valve procedures, theand 10-year survival rates are considerably improved—

tter than 80% to 90% 10-year survival rates can bepected (76,139). Sadly, even young patients with AoD havedismal 5-year survival rate of 50% after surgery due tosidual events related to unresected dissected aorta. Thisresses the importance of treating young patients withective surgery, with a less than 1% risk of death, if theyve aortic root dilatation (75,76).Repair of TAAs is one of the most extensive and highest-

sk operations done in patients. Hence, selection of patientsr repair and workup for surgery needs to be diligentlyrformed. Furthermore, the cited results are from reports of

ngle-center experiences and therefore representative ofnters of excellence in the treatment of aortic disease. Dataom “real world” experiences have demonstrated nearlyuble the morbidity and mortality rates, especially forgh-risk indications like acute aortic dissection and thoraco-dominal aortic aneurysms, suggesting that high-risk pa-nts may have better outcomes in centers specializing in the

eatment of thoracic aortic diseases (782,783).

4.8. Postprocedural Care

.8.1. Postoperative Risk Factor Managementhe recognition and treatment of thoracic aortic diseaseovide the opportunity to engage the patient in long-termrdiovascular risk factor management. Conditions such asrtic atherosclerosis and aortic aneurysm are recognized asgh-risk states by the National Cholesterol Education Pro-am, Adult Treatment Panel III and require maximal inten-ty therapy (784). In these patients, the risk of a fatal ornfatal MI is higher than the risk of amputation or aortic
pture. The risk factors with clinical trial evidence of benefit si
clude hypertension, dyslipidemia, and cigarette smokingee Section 9.2.1).

.8.2. Recommendations for Surveillance of Thoracicortic Disease or Previously Repaired PatientsASS IIa

Computed tomographic imaging or magnetic resonance imag-ing of the thoracic aorta is reasonable after a Type A or B aorticdissection or after prophylactic repair of the aortic root/ascending aorta. (74) (Level of Evidence: C)Computed tomographic imaging or magnetic resonance imag-ing of the aorta is reasonable at 1, 3, 6, and 12 monthspostdissection and, if stable, annually thereafter so that anythreatening enlargement can be detected in a timely fashion.(Level of Evidence: C)When following patients with imaging, utilization of the samemodality at the same institution is reasonable, so that similarimages of matching anatomic segments can be compared sideby side. (Level of Evidence: C)If a thoracic aortic aneurysm is only moderate in size andremains relatively stable over time, magnetic resonance imag-ing instead of computed tomographic imaging is reasonable tominimize the patient’s radiation exposure. (Level of Evi-dence: C)Surveillance imaging similar to classic aortic dissection isreasonable in patients with intramural hematoma. (Level ofEvidence: C)

he mean rate of growth for all thoracic aortic aneurysms isproximately 1 mm/y, but that growth rate increases withcreasing aneurysm diameter. Growth rates tend to be fasterr aneurysms involving the descending versus the ascendingrta, for dissected versus nondissected aortas, for those witharfan syndrome versus those without (375), and for thoseith bicuspid versus those with tricuspid aortic valves (785).he frequency of surveillance imaging is not clear as there are

data to accurately dictate surveillance intervals. It seemsudent to obtain an initial follow-up imaging study beforescharge; at 1, 3, 6, and 12 months postoperatively; and thennually after a thoracic aortic aneurysm is first detected.ssuming the aneurysm is stable in size on the first follow-upudy, repeat imaging can reasonably be repeated on annual basis. For relatively small aneurysms that are stable

om year to year on annual imaging, the writing committeelieves that an imaging frequency of every 2 to 3 years,pecially in older patients, is currently reasonable (Ta-e 17).The writing committee believes that the anatomical detailovided by CT may be better than that for MR in manystances. However, for surveillance of stable and moderateoracic aortic aneurysms, MR provides adequate informationd avoids the potential problems associated with repeateddiation exposure of CT angiography.Dissected aortas also tend to dilate progressively over time

75,786). Therefore, those with a Type B AoD managededically or those with a Type A AoD with a persistent distalssection following ascending aortic repair must also undergoriodic surveillance imaging to monitor the affected aorta forrther dilatation. When the dissected aorta is relatively stable in
ze, annual surveillance imaging is usually sufficient. The most

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oximal portion of the descending thoracic aorta, just beyonde ostium of the left subclavian artery, is most prone to bothrly and late dilatation. If this segment expands to 6.0 cm oreater or if there is rapid growth, intervention or an open repairay become necessary. Predictors of progressive dilatation orpture of a dissected aorta include complete patency of the falsemen (787) and a large false lumen size (788). Those withronic AoD are also at risk for a second acute dissection arising

om the chronic dissection itself or de novo from undissectedrta.Those with IMH are also at risk of late complications,

cluding conversion to a classic AoD and progressive aorticlatation. Conversely, in other cases there may be progres-ve reabsorption of the IMH, and in time the radiographicpearance of the thoracic aorta normalizes. While this is aore favorable outcome, such patients remain at increasedsk of late complications as the apparently healed aorta isone to the development of “ulcer-like projections andccular aneurysm,” which are associated with an increasedsk of late aneurysm formation and rupture (297). Otherctors that predict adverse events are age greater than 70ars (297), a maximum aortic diameter of 40 mm or greater,d a maximum aortic wall thickness of 10 mm or greater98). The natural history of PAUs remains poorly defined.ertainly those who present with symptoms of an acute aorticndrome are likely to have increased risk of aortic rupture,hereas those without symptoms whose ulcers are discoveredcidentally are more likely to have a chronic or slowlyowing ulcer. Those with uncomplicated ulcers are often

ble 17. Suggested Follow-Up of Aortic Pathologies Afterpair or Treatment

thology Interval Study

utesection

Before discharge, 1 mo, 6mo, yearly

CT or MR, chest plusabdomen TTE

ronicsection

Before discharge, 1 y, 2to 3 y

CT or MR, chest plusabdomen TTE

rtic rootpair

Before discharge, yearly TTE

R pluscending

Before discharge, yearly TTE

rtic arch Before discharge, 1 y, 2to 3 y

CT or MR, chest plusabdomen

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Before discharge, 1 mo, 2mo, 6 mo, yearly Or 30

days*

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ute IMH/PAU Before discharge, 1 mo, 3mo, 6 mo, yearly

CT or MR, chest plusabdomen

*US Food and Drug Administration stent graft studies usually requiredfore discharge or at 30-day CT scan to detect endovascular leaks. If there isncern about a leak, a predischarge study is recommended; however, the riskrenal injury should be borne in mind. All patients should be receiving beta

ockers after surgery or medically managed aortic dissection, if tolerated.apted from Erbel et al (539).

AVR indicates aortic valve replacement; CT, computed tomographic imaging;R, chest x-ray; IMH, intramural hematoma; MR, magnetic resonanceaging; PAU, penetrating atherosclerotic ulcer; and TTE, transthoracichocardiography.

eated medically with antihypertensive medications and tio

ose monitoring with serial imaging studies, similar to theanagement of a patient with a distal AoD (789).

5. Nursing Care and Patient/Familyducation

5.1. Nursing Care of Medicallyanaged Patientsursing care for patients with thoracic aortic disease requiresucation to ensure that both patient and family understande disease process, the importance of therapy includingntrol of hypertension and other risk factors (790,791), ande need for continued follow-up, including surveillanceaging. Because some aortic diseases are hereditary,llow-up might also include family member screening andunseling (792). For patients with acute aortic syndromes,mediate control of hypertension and pain, as well as

peated assessments of symptoms and hemodynamic status,e key elements (345).Patients transported from smaller hospitals to larger onese often alone, without the immediate support of theirmilies, and will be dependent on nursing personnel forotional support and reassurance, as well as clinical and

iritual care. Family members need to have their questionsswered honestly and in a way they can understand, to betified of any changes in the patient’s condition, and to havecess to their loved one(s) (793).

5.2. Preprocedural Nursing Careurses play a key role in answering questions that remainter initial explanations by physicians. Table 18 outlinespics that should be discussed as part of informed consentior to an endovascular or open surgical procedure.

5.3. Postprocedural Nursing Caretients with a lower acuity level after thoracic aorticocedures, including endovascular repair, are often admitteda postanesthesia care unit for initial recovery and then to antensive care unit that can provide frequent monitoring oftal signs, peripheral pulses, urine output, and neurologicalatus including lower extremity motor strength and sensa-n. Assessment of the skin in the extremities and lower torsoperformed to look for cyanotic discoloration and skin

mperature changes, which may be signs of embolization94). Embolization to cerebral vessels is another potentialsk of these procedures, so frequent monitoring of mentation,vel of consciousness, vision, speech, motor strength, andnsory function in the extremities is required. Thromboem-li to mesenteric, renal, and peripheral vascular beds may

ad to complaints of abdominal pain or the presence ofoody diarrhea or vomiting. Flank pain with changes in urinetput might indicate potential renal infarction from throm-emboli.The initial postprocedural management focuses on bloodessure control. Hypertension can result in stent migrationd bleeding from aortic suture lines. Hypotension can beually problematic with the potential to impair renal func-
n and spinal cord perfusion pressure. Hypotension presents

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risk to spinal cord perfusion pressure and requires treatmentith fluids or vasopressors. The risk of inadequate spinal cordrfusion is spinal cord ischemia, dysfunction, and potentialraplegia. Any decrease in lower extremity function must beported to the physician team immediately as it couldpresent the onset of early and potentially reversible paral-is. A typical assessment consists of asking patients tomonstrate motor strength holding their extended, straightgs off the bed one at a time for at least 5 seconds (781), withpeated assessments performed frequently for the first hoursd days after an endovascular or open procedure. Anjective scale that can be useful is outlined in Table 19. CSFains are used for many patients undergoing endovasculard surgical procedures, and the nursing care is criticallyportant (see Section 14.4).Any new back or chest pain warrants further investigationrule out the possibility of myocardial ischemia or infarctionwell as a potential endoleak. New-onset pain of this nature

quires notification of the physician team for further evalu-ion.A phenomenon associated with stent graft placement isstimplantation syndrome, which can occur within 24 hours

ble 18. Topics of Discussion for Preoperative Instructiond Informed Consent

arification of the intervention or surgical procedure

ngth of the intervention or surgical procedure

tential complications

Endovascular leak

Stroke

Paralysis

Respiratory dysfunction/failure

Renal dysfunction/failure

Myocardial infarction (especially if known coronary disease)

eoperative preparation for elective procedures

Smoking cessation

Antiplatelet or anticoagulation adjustment

Preoperative testing and instructions for obtaining tests

Chlorhexidine showers or other skin preparation

ensive care stay and environment

What to expect: monitoring, staffing ratios, equipment

Length of stay

Visiting restrictions if any

Intravascular access

Central venous lines

Arterial lines

Ventilator support and weaning

Lumbar drains

Other lines and tubes

Pain management

ansitional care unit transfer

What to expect: monitoring, staffing ratios

Length of stay

tivity progression

ernal and other activity/lifestyle precautions

rable power of attorney for health care

placement of the graft. It is characterized by fever,

ukocytosis, and occasionally thrombocytopenia. The symp-ms, which usually resolve within 1 week, are managed withalgesics and anti-inflammatory agents (794). Heparin-duced thrombocytopenia can be another source for throm-embolic events and may require treatment.Routine assessment of the cannulation site used to deploye graft is necessary. Large sheaths are used to deliver stentsd can traumatize and occlude the femoral or iliac artery.

he postoperative period presents an optimal opportunity tosess and manage cardiovascular risk factors and to educatee patient and family on the benefits of risk-reductioneasures. The AHA/ACC Guidelines for Secondary Preven-n for Patients With Coronary and Other Atherosclerotic

ascular Disease (409) outlines guides to risk factor assess-ent, patient education, and possible interventions (see theline-only Data Supplement).Patient and family preparation for discharge includesphasis of the importance of medication compliance, inci-

on care, and the need for follow-up. They must be madeare of the signs and symptoms of infection, such as

dness, swelling, drainage, and fever. Unusual or severein, change in motor strength or sensation in the extremities,d sudden weakness or dizziness, which may be symptomsnew-onset bleeding or changes in spinal cord perfusion,

ould be promptly reported.

5.4. Nursing Care of Surgicallyanaged Patientstients who have undergone open repair of aortic aneurysmsdissection will require more intensive care. A distinguish-

g feature will be whether the patient had an ascending aorticch, or descending thoracic aortic repair. Patients who haved a dissection or an aneurysm of the ascending aorta or archill have a median sternotomy incision. Patients undergoingrtic arch procedures may also have an incision over theillary artery site. Cardiopulmonary bypass is required forese procedures and can be associated with fluid retention,ectrolyte abnormalities, coagulopathies, and hypothermia.addition, patients with aortic arch repairs are subjected to

tervals of circulatory arrest and retrograde or selectiverebral perfusion. These adjuncts can result in neurologicsfunction in the postoperative period.Patients with Type B aortic dissections or descendingoracic aneurysms will have lateral thoracic or thoracoab-minal incisions. These incisions are often extensive.These patients will often have lumbar drains that have beenaced to monitor CSF pressure and CSF fluid drainage. Theessure used as a target for drainage varies but will generally

ble 19. Lower Extremity Motor Function Assessment Scale

No movement

Flicker of movement

Able to bend knee to move leg

Unable to perform straight leg raise against gravity, but better legmovement

Normal movement with expected later or demonstrated ambulation

Note: A score of �3 may be an indication for a neurological evaluation.
Adapted from Svensson et al (781).
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around 10 mm Hg. Pressures are checked hourly or moreequently in the early postoperative period. Care must beken to prevent drainage that is too rapid or too excessive toevent subdural hematoma formation (746). Any change invel of consciousness or onset of irritability, confusion,adache, or pupillary reactivity requires immediate notifica-n of the physician team and clamping of the drain.

eurologic monitoring also involves assessment of lowertremity motor and sensory function. Any deterioration maydicate inadequate spinal cord perfusion.Standard postoperative care of these patients includesntilator adjustments, optimization of blood volume, coag-ation parameters, and monitoring of other organ systemnctions (795–799). Following the transfer of patients frome intensive care unit, nursing care is focused on painanagement, progression to independent ambulation, pulmo-ry physiotherapy and incentive spirometry, and woundre. Preparation for discharge is similar to that described forwer-activity patients earlier in this section.

6. Long-Term Issues

6.1. Recommendation for Employment andifestyle in Patients With Thoracicortic DiseaseASS IIa

For patients with a current thoracic aortic aneurysm or dissec-tion, or previously repaired aortic dissection, employment andlifestyle restrictions are reasonable, including the avoidance ofstrenuous lifting, pushing, or straining that would require aValsalva maneuver. (Level of Evidence: C)

stablishing clear lifestyle goals for patients with thoracicrtic disease is important in improving long-term health andducing the risk of complications. Because regular aerobicercise, a low-fat and low-salt diet, and achieving an idealdy weight are tied to the ability to effectively control bloodessure, cholesterol, and associated aortic wall stress, pro-ding patients with clear lifestyle targets is important.voidance of tobacco is critical because it is linked to thevelopment of thoracic aortic disease and to aortic rupture.ot using cocaine or other stimulating drugs such as meth-phetamine is important as sudden surges in blood pressure

d pulse attributed to such agents have been described as aigger for aortic catastrophes (800).The prescription of exercise represents a dilemma in theanagement of patients with thoracic aortic disease. Becauseis thought that the sudden increases in dP/dt and systemicood pressure associated with physical and mental stressay be a trigger for AoD in many patients, the concept ofoiding such stresses makes sense (801). However, main-ining a regular routine of aerobic exercise has day-to-daynefits in helping patients achieve an ideal blood pressure,art rate, and body weight. Moreover, many patients simplyjoy engaging in sports such as tennis, basketball, golf, bike

ding, etc. and wish to continue in such activities if at all
ssible. th
There are no outcomes data, and scant data of any varietyr that matter, to indicate how much exercise is safe orneficial for patients with thoracic aortic disease. However,robic exercise, sometimes referred to as dynamic exercise,associated with only a modest increase in mean arterial

essure (802), and AoD rarely occurs during aerobic exer-se. Consequently, most experts believe that aerobic exer-se, particularly when heart rate and blood pressure are wellntrolled with medications, is beneficial overall. Neverthe-ss, if patients wish to engage in vigorous aerobic exercise,ch as running or basketball, one might consider performingsymptom-limited stress test to ensure that the patient doest have a hypertensive response to exercise.Conversely, with isometric exercise, there is a significantcrease in mean arterial pressure. When the Valsalva ma-uver is used for the lifting of heavy weights, there is aperimposed increase in intrathoracic pressure, followed bydramatic increase in systemic arterial pressure (802), withstolic pressures reaching 300 mm Hg or greater (803). As asult, most experts believe that heavy weight lifting ormpetitive athletics involving isometric exercise may triggeroD and/or rupture and that such activities should be avoided04). Working with patients on an individualized basis toreamline these goals based on insufficient data can beallenging. For patients who are very much interested inaintaining some sort of weight lifting program, choosingts of repetitive light weights appears to make more sensean permitting heavy weight lifting (802). For example,stead of bench-pressing 200 pounds, one might recommendlecting much lighter weights in repetitive sets to minimizee hemodynamic consequences. Patients often ask exactlyw much weight is permissible to lift. Unfortunately, it ist possible to provide a blanket answer to that question, asall depends on the patient’s size, muscular strength,ysical fitness, and how the weight is actually lifted. Ratheran try to define a numerical limit, it may be useful toplain that patients can lift whatever weight they canmfortably lift without having to “bear down” or performe Valsalva maneuver.In addition to the physiologic stress of exercise, certainorts, recreational activities, or sudden stress or trauma toe thorax can potentially precipitate aortic rupture and/orssection. Thoracic stress or trauma can occur during com-titive football, ice hockey, or soccer or may result from aiing accident, a fall while water skiing, etc. Therefore,perts often advise patients with thoracic aortic disease tooid these types of sports (805). Furthermore, rapid chesttational movement while straining or breath holding (Val-lva maneuver) may be a common denominator in manytients who develop aortic dissection (i.e., basketball, tennis,lf, baseball bat swing, chopping wood with an ax, shovel-g snow, and rapidly lifting heavy objects).In addition to the importance of setting clear lifestyle goals

ith patients with thoracic aortic disease, it is wise tophasize the importance of adherence to their medications,

pecially to beta blockers and other antihypertensive agents.tients who suddenly discontinue their medications because
ey fail to obtain a refill or perhaps forget their medications

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home when traveling, may find themselves in a hyperten-ve crisis with a potentially catastrophic result.Even patients who are compliant with their medicationsay find that their blood pressure may fluctuate betweenutine visits to their physician, resulting in months ofcessive hypertension. Therefore, patients may achieveore consistent control of their hypertension if they regularlyack their heart rate and blood pressure with a homeonitoring system. In addition, by regularly tracking theireight and activity profile, they can provide their physiciand other care members with accurate data with which toake adjustments in medication and lifestyle going forward.In terms of work, patients with thoracic aortic diseasenerally can function normally in most types of occupations.

he exception is any job involving heavy physical andanual labor accompanied by extreme isometric exercise.g., lifting heavy boxes in a stockroom, carrying furniture

and down stairs). As with the heavy weight liftingscribed earlier, this type of unusual sudden stress on therta may predispose to a triggering of either aortic rupture oroD (806). Therefore, when patients have a vocation inhich such extreme lifting might be required, it is importantdiscuss the details of their daily job responsibilities and toescribe avoidance of activities that might put them at risk.some cases patients can readily avoid such heavy lifting on

e job, but in many cases a letter from a physician explaininge restrictions may be required.Finally, patients with thoracic aortic disease should recog-ze that aortic disease is usually a lifelong condition that putsem at future risk for acute aortic syndromes. Even thoseho have received advanced surgical or endovascular therapyust understand that their aortic disease has not been “cured”

the interventions. Educating them about what to do in theent of the sudden onset of chest, back, or abdominal pain ore sudden development of an ischemic complication (i.e.,urological or limb) and the critical nature of getting to anergency department promptly is of the utmost importance.

milarly, those who live with or care for such patients shouldderstand what action needs to be taken should concerningmptoms arise.

7. Institutional/Hospitaluality Concerns

7.1. Recommendations for Qualityssessment and Improvement for Thoracicortic DiseaseASS I

Hospitals that provide regional care for patients with acutesequelae of thoracic aortic disease (e.g., procedures for tho-racic aortic dissection and rupture) should participate instandardized quality assessment and improvement activities,including thoracic aortic disease registries. Such activitiesshould include periodic measurement and regional/nationalinterfacility comparisons of thoracic aortic disease–relatedprocedural volumes, complications, and risk-adjusted mortality
rates. (Level of Evidence: C) ad
Hospitals that provide regional care for patients with acutesequelae of thoracic aortic disease (e.g., procedures for tho-racic aortic dissection and rupture) should facilitate and coor-dinate standardized quality assessment and improvement ac-tivities with transferring facilities and emergency medicalservices teams. Such activities might include:a. Cooperative joint facility meetings to discuss opportunities

for quality improvement andb. Interfacility and emergency medical services team compar-

isons of pretransfer care based on available outcome dataand future performance measures developed in accordancewith this guideline. (Level of Evidence: C)

uality assessment of outcomes for thoracic aortic diseases been ongoing. Creation of one or more standardizedoracic aortic disease registries may significantly improvepacity for quality assessment and provide outcome resultsd meaningful performance benchmarks. IRAD is an exam-e of such a registry and consists of 12 large referral centers6 countries and contains information on 290 variables,

cluding demographics, history, physical findings, manage-ent, imaging studies, and outcomes (227). Commitment tota collection, analysis, measurement, validation, and re-rting should be factored into the payment structures, as allthis will consume time, effort, and infrastructure to do it

ell (47).Moderate evidence supports “evidence-based referral”—at is, limiting specific procedures only to hospitals andysicians with experience, expertise, and capacity to takere of complex problems, especially those requiring special-ed surgical skills and support teams. To date, there is noplicit evidence supporting such a recommendation forute thoracic aortic diseases (807), although this evidenceists for infrarenal aortic surgery (808–811). Data courtesythe UHC Clinical DataBase/Resource Manager for 2006q4

rough 2007q3 indicates that for patients with:

Ruptured thoracic aortic aneurysm:30% transferred from a different hospital34% were admitted from the emergency department19% referred from a clinic or physician’s office14% transferred from a skilled nursing facility or another

nonhospital facility3% other

Thoracic AoD:28% transferred from a different hospital35% were admitted from the emergency department23% referred from a clinic, physician’s office, or health

maintenance organization9% transferred from a skilled nursing facility or another

nonhospital facility5% other

Performance measures and quality metrics (811a) haveen developed to evaluate quality of care for many areas ofrdiac and vascular disease (5,812–814), but none have yeten established for patients with thoracic aortic diseases.ssible domains of quality to assess could include proce-ral volumes (facility and operator), outcomes (e.g., risk-
justed mortality, readmission, complications), time to di-

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nosis and intervention, and structural measures (e.g., dis-nce to nearest referral center, services available, and con-gency planning) (815). The well-established evidence baser the regionalization of care for STEMI patients (816) mayrve as a model for similar initiatives for patients withoracic aortic disease (Table 20).Claims have been made that some centers either do or willit referrals of critically ill patients that may adversely

pact hospital mortality rates, although there are no discreteta to confirm or disprove this behavior (817). Hence,nchmarking and profiling efforts must recognize and ac-unt for the most physiologically precise severity-of-illnessformation and accurate and appropriate case-mix adjust-ents; otherwise, referral centers may have an incentive tofuse care for patients who could benefit from being trans-rred to their facility (818).Effective medical record systems are being developed andn enhance communication and provide caregivers withear documentation of a patient’s course and medications, asell as plans for future (819,820).

7.2. Interinstitutional Issuescute AoD or other acute problems involving any portion ofe aorta are life-threatening conditions and require placinge patient in a location where all appropriate diagnostic anderapeutic measures are available. The minimum require-ents for the care of patients with acute AoDs includeaging with CT, echocardiography, and angiography; theailability of cardiovascular surgery including cardiopulmo-ry bypass and endovascular interventions; an intensive caretting that allows continuous monitoring of blood pressured intravenous management of blood pressure; and physi-ans with personal experience and expertise in the manage-ent of patients with acute AoD (820).Transfer of patients with acute AoD from one institution toother represents a period of danger to the patient and mustplanned and carried out efficiently. It is incumbent on the

ble 20. Standardized Transferring Facility Assessment,mmunication, and Documentation for the Following Domains

Blood pressure control for hypertension

Heart rate control for tachycardia

Hemodynamic instability

Blood volume

Cardiac ischemia

Neurologic ischemia

Renal function

Mesenteric ischemia

Peripheral arterial pulses and perfusion

Activation of receiving team

Imaging expectations and communications

Timeliness and efficiency

EMS characteristics of transferring facility, including requisite personnel,requisite in-transport equipment, including catastrophic resuscitationcapabilities, in-transfer contingency planning, weather conditions,estimated transfer time, etc.

EMS indicates emergency medical services.

ansferring institution to provide prior physician-to- m

ysician communication, to stabilize and maintain bloodessure control throughout transport, and to send copies ofaging studies (821).Because patients with acute thoracic aortic diseases presentany emergency department or primary care setting, there

e opportunities for retrospective and comprehensive qualityview. Typical areas of quality assessment could include thealuation of timely detection in centers that typically are notuipped to manage such patients.Retrospective evaluation of patients with thoracic aorticseases, especially those for whom diagnosis was delayed orissed, is critical for learning about how best to more rapidlyd effectively identify future patients with life-threateningoracic aortic aneurysm and/or dissection. Independent ex-rnal review of poor outcomes may be necessary andpropriately constructed using de-identified clinical recordsthat bias is not introduced into the evaluation process.With the dissemination of this guideline, it is hoped thatese practice standards can form the basis for more wide-read use of a data registry for quality assessment andprovement for patients with thoracic aortic disease. Theals of such a data system would be to establish an extensivepulation of thoracic aortic disease patients with the intentevaluating clinical effectiveness. Domains could include

lume and outcome relationships, process of care patterns,velopment of standardized performance measures (struc-re, process, and outcomes), and facility and operator feed-ck quality data systems. It may also be possible to useisting data that use relevant ICD-9 codes (Table 2A and) and appropriate risk-adjusted methods to evaluate mor-

lity and complications.

8. Future Research Directionsnd Issues

he writing committee believes that there are many opportu-ties for additional meritorious research in thoracic aorticseases. Emerging research appears promising for the fol-wing areas, in addition to much needed research in othereas.

8.1. Risks and Benefits of Currentaging Technologies

here is a great need to balance the rapid identification oftients with acute AoD or rupture, and the relatively infre-ent occurrence against the potential risks of radiationposure and contrast toxicity incurred by current imagingethods, for the millions of patients who present with chest,ck, and/or abdominal pain. The rapid and correct diagnosisacute thoracic aortic diseases must not impose delays in

eatment of patients with acute MI. Additionally, screeningd serial radiographic imaging of younger patients clearlyposes those individuals to a risk of later radiation-relatedseases. Clinical studies of safety and efficacy of screeningotocols using current imaging methods to correctly identifytients who will benefit from surgical, endovascular, and/or
edical intervention are needed.

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8.2. Mechanisms of Aortic Dissectionhe year 2010 is the 250th anniversary of the death, attrib-able to AoD, of King George II of England, whose lastoments were memorably described by his valet (822).lthough a number of conditions are known to be associatedith dissection, such as hypertension and old age, the cause

spontaneous AoD is still unknown. Is an intimal tearllowed by a dissecting hematoma (823), or does an intra-ural hemorrhage ruptures into the lumen causing an intimalar? (824,825) In approximately 4% to 10% of dissections,

aortic intimal tear is not found. Bleeding from the vasasorum is hypothesized to be responsible for the intramuralmorrhage, but little is known about the microstructure ofe vasa vasorum walls; changes in the vasa vasorum insponse to aging, hypertension, and inflammation; and rela-n of extracellular and intracellular metalloproteinases tootein turnover in the walls of the vasa vasorum. Researchto these questions may shed light on the pathogenesis ofssection and provide opportunities for prevention and treat-ent.

8.3. Treatment of Malperfusion andeperfusion Injuryalperfusion of the gut, spinal cord, kidneys, and lowertremities doubles the mortality of AoD (247,826). New

eatment strategies for malperfusion and reperfusion injurye needed to improve the dismal prognosis of patients withute dissection and malperfusion syndromes.

8.4. Gene-Based Mechanisms and Models

.4.1. Aortic Disease Management Based on thenderlying Genetic Defectss genes are identified leading to an inherited predispositionr thoracic aortic disease, it is becoming increasingly evidentat the clinical management of thoracic aortic disease asso-ated with different gene mutations may differ. For example,utations in TGFBR1 and TGFBR2 predispose patients tortic dissection at an aortic diameter of 5.0 cm or less,ading to the recommendation that the aorta be replaced atameters as small as 4.0 cm. As genes are identified that canuse predisposition to thoracic aortic disease, clinical studiese needed on these patients to determine their optimalanagement.

.4.2. Biomarkers for Acute Aortic Dissectionquick laboratory test with at least a high sensitivity, if notecificity, could lead to fewer missed or delayed diagnoses

acute AoD. None currently exists. D-dimer has beenoposed as a screening test for acute AoD (see Section6.1.2). A prospective study regarding the safety and efficacysuch a strategy is needed.

.4.3. Genetic Defects and Molecularathway Analysesecent studies in the mouse model of Marfan syndrome ande identification of TGFBR1 and TGFBR2 mutations sug-sts that alteration of TGF-Beta signaling is involved. Theentification of mutations in 2 components of the contractile
it in smooth muscle cells, beta-myosin heavy chain and te
pha actin, has indicated a role of maintenance of smoothuscle cell contractile function in preserving aortic structured preventing these aortic diseases. Further studies identi-ing defective genes and analyzing pathways using humansues or mouse models will be the basis for understandinge molecular pathology and will be the first step towardvelopment of rational medical therapies for thoracic aorticsease.

.4.4. Clinical Trials for Medical Therapy forortic Aneurysmsecent studies in the mouse model of Marfan syndrome withrtic disease similar to that seen in humans showed the

eatment with losartan normalized aortic root growth andrtic wall architecture (6). Doxycycline, an inhibitor ofMP, significantly delayed aneurysm rupture in this mouseodel. A clinical trial is ongoing in patients with Marfanndrome under the age of 25 years to determine if losartanlays aortic progression. More aggressive trials (otherents, 2 or 3 agent therapies) based on the results in mouseodels to prevent the onset of aortic disease in individualsnetically predisposed or to delay the enlargement of al-ady formed aneurysms are needed.

8.5. Aortic Atheroma and Atherosclerosisentification and Treatment

s noted in Section 11, there is a need for research into theechanistic aspects of aortic arch atheroma in causingbolic events. Additionally, for those patients with known

rtic arch atheroma, a randomized blinded controlled trial iseded to test currently available treatment options, bothedical and surgical, to prevent embolic vascular events.The value of current imaging (MR, CT, TEE) technologiesidentify and quantitate risk for patients with aortic arch

heroma is unknown.

8.6. Prediction Models of Aortic Rupturend the Need for Preemptive Interventionse need to have models or indices that are better than justrtic diameter to predict rupture and to better determine thest timing for surgical or endovascular intervention. Sincee advent of CT and MR scanning, a massive amount ofdiological data has accumulated with multiple studies individual patients that could be linked to clinical data anded for this purpose. The evidence base regarding theinical course of patients with Marfan syndrome is morebust than for patients with degenerative aneurysms andher thoracic aortic conditions. An ongoing registry similarthat used for patients with Marfan syndrome would be a

ace to start.

8.7. National Heart, Lung, and Bloodstitute Working Group Recommendations

he National Heart, Lung, and Blood Institute Workingroup on Research in Marfan Syndrome and Related Disor-rs (827) posted a summary of recommendations for futuresearch which apply to the broad range of patients withoracic aortic disease. The importance of multidisciplinary
ams and collaborative research models were stressed:

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Existing registries should be expanded or new registriesdeveloped to define the presentation, natural history, andclinical history of aneurysm syndromes.Biological and aortic tissue sample collection should beincorporated into every clinical research program onMarfan syndrome and related disorders and funds shouldbe provided to ensure that this occurs. Such resources,once established, should be widely shared among investi-gators.An Aortic Aneurysm Clinical Trials Network should bedeveloped to test both surgical and medical therapies inpatients with thoracic aortic aneurysms. Partnership in thiseffort should be sought with industry, academic organiza-tions, foundations, and other governmental entities.The identification of novel therapeutic targets and biomar-kers should be facilitated by the development of geneti-cally defined animal models and the expanded use ofgenomic, proteomic and functional analyses. There is aspecific need to understand cellular pathways that arealtered leading to aneurysms and dissections, and todevelop robust in vivo reporter assays to monitor TGF-Beta and other cellular signaling cascades.The developmental underpinnings of apparently acquiredphenotypes should be explored. This effort will be facili-tated by the dedicated analysis of both prenatal and earlypostnatal tissues in genetically defined animal models andthrough the expanded availability to researchers of surgicalspecimens from affected children and young adults.

The writing committee enthusiastically endorses thesencepts.

taff

merican College of Cardiology Foundation

hn C. Lewin, MD, Chief Executive Officer

harlene May, Senior Director, Science and Clinical Policy

isa Bradfield, CAE, Associate Director, Science and Clini-cal Policy

ark D. Stewart, MPH, Associate Director, Evidence-BasedMedicine

e Keller, BSN, MPH, Senior Specialist, Evidence-BasedMedicine

rin A. Barrett, Senior Specialist, Science and Clinical Policy

sse M. Welsh, Specialist, Science and Clinical Policy

merican Heart Association

ancy Brown, Chief Executive Officer

ayle R. Whitman, PhD, RN, FAHA, FAAN, Senior VicePresident, Office of Science Operations

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7. Furnary AP, Gao G, Grunkemeier GL, et al. Continuous insulin infusionreduces mortality in patients with diabetes undergoing coronary arterybypass grafting. J Thorac Cardiovasc Surg. 2003;125:1007–21.

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0. Eagle KA, Isselbacher EM, DeSanctis RW. Cocaine-related aorticdissection in perspective. Circulation. 2002;105:1529–30.

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2. Williams MA, Haskell WL, Ades PA, et al. Resistance exercise inindividuals with and without cardiovascular disease: 2007 update: ascientific statement from the American Heart Association Council onClinical Cardiology and Council on Nutrition, Physical Activity, andMetabolism. Circulation. 2007;116:572–84.

3. Palatini P, Mos L, Munari L, et al. Blood pressure changes duringheavy-resistance exercise. J Hypertens Suppl. 1989;7:S72–3.

4. Hatzaras I, Tranquilli M, Coady M, et al. Weight lifting and aortic
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6. Elefteriades JA, Hatzaras I, Tranquilli MA, et al. Weight lifting andrupture of silent aortic aneurysms. JAMA. 2003;290:2803.

7. Williams OD. Quality of care versus provider volume: does one lead tothe other? Med Care. 2003;41:1127–8.

8. Hill JS, McPhee JT, Messina LM, et al. Regionalization of abdominalaortic aneurysm repair: evidence of a shift to high-volume centers in theendovascular era. J Vasc Surg. 2008;48:29–36.

9. Schermerhorn ML, Giles KA, Hamdan AD, et al. Population-basedoutcomes of open descending thoracic aortic aneurysm repair. J VascSurg. 2008;48:821–7.

0. Norgren L, Larzon T. Endovascular repair of the ruptured abdominalaortic aneurysm. Scand J Surg. 2008;97:178–81.

1. Troeng T. Volume versus outcome when treating abdominal aorticaneurysm electively: is there evidence to centralise? Scand J Surg.2008;97:154–9.

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2. Estes NA III, Halperin JL, Calkins H, et al. ACC/AHA/Physician Consor-tium 2008 clinical performance measures for adults with nonvalvular atrialfibrillation or atrial flutter. J Am Coll Cardiol. 2008;51:865–84.

3. Krumholz HM, Anderson JL, Brooks NH, et al. ACC/AHA clinicalperformance measures for adults with ST-elevation and non-ST-elevation myocardial infarction. J Am Coll Cardiol. 2006;47:236–65.

4. Thomas RJ, King M, Lui K, et al. AACVPR/ACC/AHA 2007 Perfor-mance measures on cardiac rehabilitation for referral to and delivery ofcardiac rehabilitation/secondary prevention services. J Am Coll Cardiol.2007;50:1400–33.

5. Luft HS. From observing the relationship between volume and outcometo making policy recommendations: comments on Sheikh. Med Care.2003;41:1118–22.

6. Ting HH, Yang EH, Rihal CS. Narrative review: reperfusion strategiesfor ST-segment elevation myocardial infarction. Ann Intern Med.2006;145:610–7.

7. Luft HS, Bunker JP, Enthoven AC. Should operations be regionalized?The empirical relation between surgical volume and mortality. N EnglJ Med. 1979;301:1364–9.

8. Dudley RA, Johansen KL, Brand R, et al. Selective referral to high-volume hospitals: estimating potentially avoidable deaths. JAMA. 2000;283:1159–66.

9. Jacobs AK. Regional systems of care for patients with ST-elevationmyocardial infarction: being at the right place at the right time.Circulation. 2007;116:689–92.

0. Rosenberg AL, Hofer TP, Strachan C, et al. Accepting critically illtransfer patients: adverse effect on a referral center’s outcome andbenchmark measures. Ann Intern Med. 2003;138:882–90.

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7. Dietz H, Deveraux R, Loeys BL. NHLBI working group research inMarfan syndrome and related disorders. Available at: http://www.nhlbi.nih.gov/meetings/workshops/marfan20070430.htm. Ac-cessed January 7, 2010.

EY WORDS: ACC/AHA Clinical Practice Guideline � thoracic aorticsease � thoracic aortic dissection � thoracic aortic aneurysm �tramural hematoma � genetic syndromes associated with thoracicrtic aneurysm � emergency department � acute thoracic aortic
sease presentation and evaluation.
http://www.nhlbi.nih.gov/meetings/workshops/marfan20070430.htm

http://www.nhlbi.nih.gov/meetings/workshops/marfan20070430.htm

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pendix 1. Author Relationships With Industry and Other Entities—2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVMidelines for the Diagnosis and Management of Patients With Thoracic Aortic Disease

mmitteember Employment Consultant Speaker

Ownership/Partnership/

Principal Research

Institutional,Organizational or Other

Financial Benefit Expert Witness

en F.atzka,air

Cardiac, Vascular & ThoracicSurgeons Inc. and TriHealth

Inc.—Medical Director,Cardiac Surgery

None None None None None ● 2007; Defense;Aortic Dissection

orge L.kris

University of Chicago MedicalCenter—Professor ofMedicine; Director,

Hypertension Center

● Abbott● Boehringer Ingelheim● Bristol-Myers Squibb/

Sanofi-aventis● Forest Laboratories● GlaxoSmithKline● Merck

● Forest Laboratories● GlaxoSmithKline● Merck● Novartis

None ● Forest Laboratories● GlaxoSmithKline● Myogen● National Institutes

of Health (NIDDK/NHLBI)

None None

hua A.ckman

Brigham & Women’sHospital—Director,

Cardiovascular FellowsProgram

● Bristol-Myers Squibb● Sanofi-aventis

● Bristol-MyersSquibb

● GlaxoSmithKline● Merck● Sanofi-aventis

None None None None

bert M.rsin

Seattle Cardiology—Director,Endovascular Services &

Clinical Research

● Abbott Vascular● Boston Scientific● Bristol-Myers Squibb● Cordis Endovascular● Eli Lilly● EV3● ReVascular

Theraputics● Sanofi-aventis● Vascular Solutions● W.L. Gore

● Boston Scientific● Bristol-Myers

Squibb● Daiichi Sankyo● Eli Lilly● Sanofi-aventis● The Medicines

Company

● VascularSolutions

● Boston Scientific ● Boston Scientific● Cordis Endovascular● Vascular Solutions

● Expert witness ina case involvingiatrogenic type Bdissection

cent F.rr

Uniformed ServicesUniversity of Health

Science—Professor ofMedicine

None None None None None None

nald E.sey, Jr

Atlantic Health—VicePresident of Quality & ChiefMedical Officer; Associate

Professor of Medicine, MountSinai School of Medicine


A.le

University of Michigan HealthSystem—Albion Walter

Professor of InternalMedicine; Clinical Director,

Cardiovascular Center

● NHLBI● Robert Wood Johnson

Foundation● Sanofi-aventis

None None ● Blue Cross/BlueShield

● Bristol-MyersSquibb

● National Institutesof Health

● Pfizer

None None

e K.rmann

Mount Sinai MedicalCenter—Assistant Professor

of Emergency Medicine;Director, Chest Pain Unit


c M.elbacher

Massachusetts GeneralHospital—Associate

Professor of Medicine,Harvard Medical School;

Co-Director, Thoracic AorticCenter

None None None None None ● 2007; Plantiff;Aortic Dissection

A.zerooni

University of Michigan HealthSystem—Professor of

Medicine; Director,Cardiothoracic Radiology

● GE Healthcare● Vital Images

None None None ● GERRAF (GERadiology ResearchFellowship)

None

holas T.uchoukos

Missouri Baptist MedicalCenter—Cardiovascular

Surgeon

● Edwards Lifesciences None None None None ● 2006; Defense;Aortic Dissection

ce W.le

The Cleveland Clinic—Chair,Heart and Vascular Institute


nna M.ewicz

University of TexasSouthwestern Medical

Center—President GeorgeH.W. Bush Chair in

Cardiovascular Medicine;Professor & Director, Division

of Medical Genetics

None None None ● Doris DukeFoundation

● Genetech● National Institutes

of Health● Vivian Smith

Foundation

None None

(Continued)

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pendix 1. Continued

mmitteember Employment Consultant Speaker


Principal Research



vid L.ich

Mount Sinai MedicalCenter—Professor & Chair,

Department ofAnesthesiology


uvik Sen University of South CarolinaSchool of

Medicine–Professor andChair, Department of

Neurology

● Coaxia● Bristol-Myers Squibb● Pfizer● Sanofi-aventis

● BoehringerIngelheim

None ● American HeartAssociation

● Genetech● Sanofi-aventis

None None

ie A.inn

Stanford University School ofMedicine—CardiovascularClinical Nurse Specialist


s G.nsson

The ClevelandClinic—Director, The Centerfor Aortic Surgery; Director,

Marfan Syndrome andCollective Tissue Disorder

Clinic

None None None ● EdwardsLifesciences

● Evalve

None None

vid M.liams

University of Michigan HealthSystem—Professor,

Department of Radiology;Director, Interventional

Radiology

● W.L. Gore None None ● W.L. Gore● Medtronic

None ● 2000; Defense;Failure todiagnose andtreat mesentericischemia withaortic dissection

● 2009; Defense;Failure todiagnose andtreat mesentericischemia withaortic dissection

NHLBI indicates National Heart, Lung, and Blood Institute; NIDDK, National Institute of Diabetes and Digestive and Kidney Diseases.This table represents the relevant relationships of committee members with industry and other entities that were reported orally at the initial writing committee

eeting and updated in conjunction with all meetings and conference calls of the writing committee during the document development process. It does not necessarilyflect relationships with industry at the time of publication. A person is deemed to have a significant interest in a business if the interest represents ownership of

or more of the voting stock or share of the business entity, or ownership of $10 000 or more of the fair market value of the business entity; or if funds receivedthe person from the business entity exceed 5% of the person’s gross income for the previous year. A relationship is considered to be modest if it is less thannificant under the preceding definition. Relationships noted in this table are modest unless otherwise noted.
*Significant (greater than $10 000) relationship.

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pendix 2. Reviewer Relationships With Industry and Other Entities—2010 ACCF/AHA/AATS/ACR/ASA/SCA/SCAI/SIR/STS/SVMidelines for the Diagnosis and Management of Patients With Thoracic Aortic Disease

rviewer Representation Consultant Speaker


Principal Research



jadahameed

Official Reviewer—Societyfor Vascular Medicine


hard A.rnstein

Official Reviewer—AmericanStroke Association


ristopherBuller

Official Reviewer—ACCF/AHATask Force Lead Reviewer


ert T.eung

Official Reviewer—Society ofCardiovascular

Anesthesiologists

● EKR Therapeutics● The Medicines

Company● Neuralstem● Schering Plough

● EKR Therapeutics● The Medicines

Company● PDL Biopharm

None ● The MedicinesCompany*

● Neuralstem*● PDL Biopharm

None None

hael D.ke

Official Reviewer—Society ofInterventional Radiologists

● W.L. Gore*● Medtronic

● Cook None ● Cook● W.L. Gore*● Medtronic

None None

tionette S.mes

Official Reviewer—AHACardiovascular Surgery and

Anesthesia Committee


bert A.yton

Official Reviewer—ACCFBoard of Trustees

● Medtronic None None None None None

ford J.vinsky

Official Reviewer—Societyfor CardiovascularAngiography and

Interventions

None None None ● Possis Corp. None None

tt Kinlay Official Reviewer—Societyfor Vascular Medicine

None ● Merck● Pfizer

None ● Pfizer None None

hard J.vacs

Official Reviewer—ACCFBoard of Govenors


ristinerangano

Official Reviewer—Society ofCardiovascular

Anesthesiologists


ven R.ss|fe

Official Reviewer—AmericanStroke Association

None ● BoehringerIngelheim

None ● American HeartAssociation*

None None

c Roselli Official Reviewer—Society ofThoracic Surgeons

● Medtronic● Vascutek

None None ● Cook None None

off D.bin

Official Reviewer—AmericanCollege of Radiology

● Fovia ● Bracco ● TeraRecon ● Biosense-Webster* None None

nk J.icki

Official Reviewer—AmericanCollege of Radiology

● Bracco● Siemens Medical● Toshiba Medical

Systems*● Vital Images

● Bracco● Siemens Medical● Toshiba Medical

Systems*● Vital Images

● SiemensMedical

● Bracco● Toshiba Medical

Systems*

None None

ralf M.ndt

Official Reviewer—AmericanAssociation for Thoracic

Surgery

None None None ● Bolton Medical ● Atricure● Bolton Medical● Jarvik Heart● Medtronic● Sorin

Group/Carbomedics● St. Jude Medical● Thoratec

Corporation● Ventracor● W.L. Gore

None

hard D.ite

Official Reviewer—AHAPeripheral Vascular Disease

Council


es P.ar

Official Reviewer—Societyfor CardiovascularAngiography and

Interventions

● Abbott Vascular● Cordis*● Medtronic Vascular

● Abbott Vascular● Cordis*● Medtronic Vascular

None ● Abbott Vascular● Cordis*● Medtronic Vascular

None None

att Decker Organizational Reviewer—American College of

Emergency Physicians


h M.sowsky

Organizational Reviewer—American College of

Emergency Physicians


ile Mohler Organizational Reviewer—American College of

Physicians


(Continued)

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pendix 2. Continued

rviewer Representation Consultant Speaker


Principal Research



es Burke Content Reviewer—ACCFCatherization Committee


ard P.en

Content Reviewer None None None None None None

rk A.ager

Content Reviewer—ACCF/AHA Task Force on Practice

Guidelines


e G. Diez Content Reviewer—ACCFCatherization Committee

● Sanofi-aventis None None None None None

n A.fteriades

Content Reviewer ● Baxter None ● Coolspine ● Celera Diagnostics None ● 2006; Plaintiff;Aortic Dissection*

Craigler

Content Reviewer ● Medtronic ● St. Jude Medical None ● NHLBI● Stanford PARTNER

Trial

khimura

Content Reviewer—ACCF/AHA Task Force on Practice

Guidelines


rick T.ara

Content Reviewer None None None None None None

rlos Ruiz Content Reviewer—ACCFInterventional Council


ACCF indicates American College of Cardiology Foundation; AHA, American Heart Association; and NHLBI, National Heart, Lung, and Blood Institute.This table represents the relevant relationships with industry and other entities that were disclosed at the time of peer review. It does not necessarily reflect

lationships with industry at the time of publication. A person is deemed to have a significant interest in a business if the interest represents ownership of 5% orore of the voting stock or share of the business entity, or ownership of $10 000 or more of the fair market value of the business entity; or if funds received bye person from the business entity exceed 5% of the person’s gross income for the previous year. A relationship is considered to be modest if it is less than significantder the preceding definition. Relationships in this table are modest unless otherwise noted. Names are listed in alphabetical order within each category of review.

*Significant (greater than $10 000) relationship.

pendix 3. Abbreviation List

A�abdominal aortic aneurysm

D�aortic dissection

D�coronary artery disease

F�cerebrospinal fluid

�computed tomographic imaging

G�electrocardiogram

A�giant cell arteritis

H�intramural hematoma

R�international normalized ratio

AD�International Registry of Acute Aortic Dissection

EP�motor evoked potential

I�myocardial infarction

MP�matrix metalloproteinase

R�magnetic resonance imaging

U�penetrating atherosclerotic ulcer

EP�somatosensory evoked potentials

FP�steady-state free precession

EMI�ST-elevation myocardial infarction

A�thoracoabdominal aneurysm

E�transesophageal echocardiogram

�transient ischemic attack

A�traumatic rupture of the aorta

E�transthoracic echocardiogram

C�University HealthSystem Consortium