Raymond LiuGillian Lieberman, MDRaymond LiuGillian Lieberman, MD May 2001

Coarctation of the AortaRaymond Liu, HMS IIIGillian Lieberman, MD

Raymond LiuGillian Lieberman, MD

Patient L.D.

• 19 y.o. female who presents with HTN

• HTN first noted at age 12 with no further work-up

• Recently took own BP at 180/120

• Has experienced frequent HA with minimal exertion

• PMH: Noncontributory

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Raymond LiuGillian Lieberman, MD

L.D. Physical Exam

• VS: Pulse: 69 BP: Right arm:145/88 Left arm: 148/90 Doppler lower extremity: 90

• Cor: Nl S1 S2. II/VI systolic ejection murmur that radiates to intrascapular region. No diastolic murmur, no S3/S4. Femoral pulses barely palpable. Lower extremity pulses nonexistant

• Rest of physical exam noncontributory

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Raymond LiuGillian Lieberman, MD

Differential Diagnosis of Pediatric HTN

• Infants• Renal artery

thrombosis • Congenital renal dz.• Coarctation of the

aorta• Bronchopulmonary

dysplasia• Patent ductus

arteriosus• IVH

•1-10yrs•Renal Disease•Coarctation of the aorta•Pheochromocytoma•Mineralcorticoid excess•Hyperthyroidism•Neurogenic tumors

•>10yrs•Renal Disease•Essential HTN

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Raymond LiuGillian Lieberman, MD

Background of Coarctation of the Aorta

•7% of children with congenital heart disease •7th most common congenital heart disease• Males > Females by factor of 2• Associated with Turner’s syndrome, Sturge-Weber

syndrome, Neurofibromatosis, and William’s syndrome• Complications include aneurysms, dissections, or

endocarditis• Unoperated coarctation has a 90% mortality by age 50

average age of death at 35 yrs.

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Raymond LiuGillian Lieberman, MD

Anatomy of Coarctation of the Aorta• Juxtaductal coarctation(Adult type)

• Narrowing of thoracic descending aorta just beyond remnant of ductusarteriosus

• 95% of all coarctation cases• Net left-to-right shunting occurs• Collaterals develop • 70% of pts have bicuspid aortic valve

• Tubular hypoplasia(Infantile type)• More diffuse narrowing of transverse

aorta and aortic isthmus• Proximal to patent ductus arteriosus

Courtesy of www.vh.org6

Raymond LiuGillian Lieberman, MD

Anatomy of Coarctation of the Aorta

NEJM Volume 342(4) 27 January 2000

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Raymond LiuGillian Lieberman, MD

Pathology of Coarctation of the Aorta

Cotran et al. Robbins Pathological Basis of Disease. 1999.

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Coarctation

Raymond LiuGillian Lieberman, MD

Embryology of Coarctation• 4th arch persists on left

to connect dorsal aortaand ventral aorta and form aortic arch

• 6th arch develops distally into ductusarteriosus

• Coarctation results from4th and 6th arch development problem

www.vh..org9

Raymond LiuGillian Lieberman, MD

Imaging Modalities

• Plain Film

•Echocardiogram

•Magnetic Resonance Imaging

•Angiography

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Raymond LiuGillian Lieberman, MD

Plain Film Imaging

• Advantages• Ease of access• Relative ease of interpretation• Sensitive

• Limitations• Nonspecific• Lack of quantitative data

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Raymond LiuGillian Lieberman, MD

Plain Film Findings

• Classic “3” sign• Rib notching of

posteriorthird of ribs 3-8

• “E” sign on esophagram

• Infantile cardiomegaly

• Infantile pulmonary congestion

Courtesy of Children’s Hospital, Boston12

Raymond LiuGillian Lieberman, MD

Classic “3” Sign

www.vh.org

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Raymond LiuGillian Lieberman, MD

Rib Notching

NEJM 1/27/00 Vol. 342, No. 4

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Raymond LiuGillian Lieberman, MD

“E” Sign on EsophogramLAO View

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Coarctation

Raymond LiuGillian Lieberman, MD

Infantile Cardiomegaly

Courtesy of Children’sHospital, Boston

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Raymond LiuGillian Lieberman, MD

Infantile Pulmonary Congestion

Courtesy of Children’s Hospital, Boston17

Raymond LiuGillian Lieberman, MD

Echocardiogram Imaging

• Advantages• First-line diagnostic imaging• Ease of accessibility• Low cost• Quantitative data: pressure gradients, LVH, anatomical

measurements

• Limitations• Limited by narrow field of view• Limited accuracy in quantitative analysis

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Raymond LiuGillian Lieberman, MD

Echocardiographic Findings

Multimedia EncylopediaOf Congenital Heart Disease, 1996

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Raymond LiuGillian Lieberman, MD

Echocardiographic Findings

www.vh.org20

Raymond LiuGillian Lieberman, MD

Echocardiographic Findings: Doppler Measurements

Courtesy of Yale School of Medicine

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Raymond LiuGillian Lieberman, MD

Bicuspid Aortic Valve

NEJM 1/27/00 Vol. 342, No. 4

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Raymond LiuGillian Lieberman, MD

Magnetic Resonance Imaging

• Advantages• Allows tomographic imaging in arbitrary anatomic

sections• Accurate quantitative data: Gradients, Anatomical

Measurements• No ionizing radiation or contrast medium needed

• Limitations• Slow scanning process• Claustrophobia is issue for children• High expense

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Raymond LiuGillian Lieberman, MD

Types of MRI

• Spin echo(SE) pulse sequences• Mainstay of morphologic diagnosis• Provides highest contrast resolution with limited temporal

resolution• Blood is black with internal structures contrasted against

signal void of heart chambers

• Gradient reversal echo(GRE) pulse sequences• Can acquire images within short cardiac time interval• Blood is white providing noninvansive contrast medium

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Raymond LiuGillian Lieberman, MD

Spin Echo MRI of Patient L.D.

Courtesy of Children’s Hospital, Boston25

Coarctation

Raymond LiuGillian Lieberman, MD

SE MRI of Infantile Coarctation• Narrowing of aortic

isthmus with multiple collaterals

Courtesy of Children’s Hospital, Boston26

Coarctation

Raymond LiuGillian Lieberman, MD

GRE MRI of L.D.

Courtesy of Children’sHospital, Boston27

Left Ventricular Hypertrophy

Raymond LiuGillian Lieberman, MD

Gadolinium Enhanced MR Angiogram

Courtesy of Children’s Hospital, Boston28

Raymond LiuGillian Lieberman, MD

3D MRI Reconstruction of L.D.

Courtsey of Children’s Hospital, Boston29

Raymond LiuGillian Lieberman, MD

MRI of Coarctation

• T1 Weighted Spin Echo

• GRE Phase Contrast Cine Study

•Contrast Enhanced 3D Reconstruction

Courtesy of Dr. Vrachiolitis, BIDMC30

Raymond LiuGillian Lieberman, MD

Angiography of Coarctation of the Aorta

• Advantages• Gold standard for visualization of lesion• Defines valvular and anatomic disease• Delineates collateral circulation• Offers opportunity for therapeutic intervention

• Limitations• Invasive procedure requiring ionizing radiation• Limited x-ray tube power and intensifier size for babies

and young children

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Raymond LiuGillian Lieberman, MD

Thereaputic Options for Coarctation of the Aorta

• Medical• Surgical• Catheterization

• Balloon angioplasty• Balloon-expandable stents

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Raymond LiuGillian Lieberman, MD

Surgical Intervention

• Resection and end-to-end anastomosis is gold standard

• Indicated for pts. with transcoarctation pressure gradient >30 mmHg

• 90% children are normotensive with 5yr f/u• Residual HTN develops in ½ of pts. >40yrs

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Raymond LiuGillian Lieberman, MD

Catheterization

• Balloon angioplasty• Indicated for both postoperative restenosis and

dilatation of native coaractation• Mortality of angioplasty ranges from 0.0-2.5%

compared with mortality of surgery from 4-25%• 50% restenosis rates• Limited by higher incidence of aortic aneurysm and

recurrent coarctation

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Raymond LiuGillian Lieberman, MD

Catheterization

• Balloon-expandable stents• Flow to major branches of aortic arch maintained

without obstruction at 3 yr f/u• 32/33 patients with native or recurrent coarctation had

complete relief of the obstruction occurred in 32. • Comparable morbidity and mortality results with

balloon angioplasty• Limited by small amount of data

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Raymond LiuGillian Lieberman, MD

Pre-Dilatation Cath for L.D.

Courtesy of Children’s Hospital, Boston

Raymond LiuGillian Lieberman, MD

Balloon Angioplasty for L.D.

Courtesy of Children’s Hospital, Boston37

Raymond LiuGillian Lieberman, MD

Post-Dilatation Catherization for L.D.

Courtesy of Children’s Hospital, Boston

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Raymond LiuGillian Lieberman, MD

Post Procedure CXR

Courtesy of Children’s Hospital, Boston39

Post-ProcedureDecrease in Dilation

Pre-ProcedureDilation

Raymond LiuGillian Lieberman, MD

Catheterization Stents

Radiologic Clinics of North AmericaVol. 37 Num. 2 3/9940

Raymond LiuGillian Lieberman, MD

Catheterization Stents

Courtesy of Yale School of Medicine41

Raymond LiuGillian Lieberman, MD

Summary

• Coarctation of the aorta is narrowing of thoracic descending aorta located distal(juxtaductal) or proximal(infantile) to remnant of ductus arteriosus

• Imaging modalities include plain film, echocardiogram, MRI, or angiography.

• Cardiac catheterization offers opportunity for both radiological interpretation and therapeutic intervention

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Raymond LiuGillian Lieberman, MD

References

Behrman. Nelson Textbook of Pediatrics. 2000.Braunwald, E. Heart Disease: A Textbook of Cardiovascular Medicine 2001Brickner, M. Elizabeth. Hillis, L. David. Lange, Richard A.. “Medical Progress: Congenital

Heart Disease in Adults: First of Two Parts. NEJM. 2000; 342: 256-263.Daniel, Werner G.. Mugge, Andreas.. “Medical Progress: Transesophageal

Echocardiography.” NEJM. 1995; 332:1268-1279. Ing, F. “Early diagnosis of coarctation of the aorta in children: a continuing dilemma”

Pediatrics 1996; 98: 378-82.Sinaiko, Alan. “Current Concepts: Hypertension in Children.” NEJM. 1996; 335:1968-

1973.www.vh.org

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Raymond LiuGillian Lieberman, MD

Acknowledgements• Michael Landzberg, Children’s Hospital• Dr. Senna, Children’s Hospital• Larry Barbaras, Cara Lyn D’amour, Webmasters• Thomas Vrachiolitis, BIDMC• Daniel Saurborn, BIDMC• Eric Niendork, BIDMC• Gillian Lieberman, BIDMC• Lynn Sosa, HMS• Yi-Bin Chen, HMS

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