Coronary Anomalies

Daniel Kramer

December 17, 2008

Inspiration I – RAO Caudal

Inspiration I – RAO Cranial

Inspiration I – LAO - RCA

Inspiration I – LAO - LCX

Inspiration II – RAO Caudal

Inspiration II – LAO Caudal

Inspiration II – F1 Hazing Shot

Inspiration II

Inspiration II – Aortic and PA Catheters

Lingering Questions

• What is normal?

• What is the risk and mechanism of sudden cardiac death in these patients?

• What modalities provide useful diagnostic or prognostic information?

• What is the optimal management for various anatomical oddities?

Agenda

• Anatomy and epidemiology

• Physiology and Risk Assessment

• Case studies

• Clinical Managment

Normal Anatomy

Grossman’s Cardiac Catheterization, Angiography, and Inervention 2006

Echocardiography: Normal RCA and LMCA

Echocardiography – Normal LCA

Cardiac MRI – Coronary Sequence

What is normal?

Lack of consensus on definitions and diagnosis

Anatomy vs physiology

Clinical significance

1% Rule?

Circulation 2007;115:1296-1305

Epidemiology

Estimates vary from 1-5%

Texas series of 1950 pts found 5.6% overall

RCA from LSV 0.92%

LCA from RSV 0.15%

Total ACAOS 1.07%

Circulation 2007;115:1296-1305

Possible pathways for ACAOS

Circulation 2007;115:1296-1305

1. Retrocardiac

2. Retroaortic

3. Preaortic / Inter-arterial

4. Intraseptal / Intramural

5. Prepulmonary

AL = antero-left

AR = antero-right

P = posterior

pulmonarypulmonarytrunktrunk

RR LL

NNnormalnormal

inter-arterialinter-arterial

pre-pulmonicpre-pulmonic

retro-aorticretro-aortic

RCARCA

Anatomic Variants

Cartoon courtesy of Dr. Fred Wu, Children’s Hospital Boston

pulmonarypulmonarytrunktrunk

RR LL

NNLMCALMCA

inter-arterialinter-arterial

Anatomic Variants

pre-pulmonic

retro-aortic

Cartoon courtesy of Dr. Fred Wu, Children’s Hospital Boston

Agenda

• Anatomy and epidemiology

• Physiology and Risk Assessment

• Case studies

• Clinical Managment

Mechanisms and Classification

Circulation 2007;115:1296-1305

Basso C. JACC 2000; 35(6):1493-501

Intermittent Ischemia

Pathophysiology of Sudden Death

Causes of Sudden Death in 387 Young Athletes

Cause no. of athletes percent

Hypertrophic Cardiomyopathy 102 26.4

Commotio cordis 77 19.9

Coronary artery anomalies 53 13.7

LV hypertrophy of indeterminate causation 29 7.5

Myocarditis 20 5.2

Ruptured aortic aneurysm (Marfan’s) 12 3.1

ARVD 11 2.8

Tunneled (bridged) coronary artery 11 2.8

Aortic stenosis 10 2.6

Premature atherosclerosis 10 2.6

Dilated cardiomyopathy 9 2.3

Long QT syndrome 3 0.8

Maron BJ. JAMA 1996; 276:199-204

• Military recruits 1977-2001 (N = 6.3 million)

• 126 nontraumatic deaths• 64 with identifiable

cardiac disease• 21 coronary artery

anomalies, all LCA from RSV

• Prodromal symptoms (chest pain, dyspnea, syncope) noted in autopsy reports of 11 cases.

Eckart et al. Ann Intern Med. 2004;141:829-834

Basso C. JACC 2000; 35(6):1493-501

Basso C. JACC 2000; 35(6):1493-501

Agenda

• Anatomy and epidemiology

• Physiology and Risk Assessment

• Case studies

• Clinical Managment

RCA from the LSV

Courtesy of Dr. Anne Marie Valente, Children’s Hospital Boston

RCA from the LSV

Courtesy of Dr. Anne Marie Valente, Children’s Hospital Boston

RCA from the LSV

Courtesy of Dr. Anne Marie Valente, Children’s Hospital Boston

LMCA from the RSV

Circulation 1974;50;780-787

LMCA from the RSV

Circulation 1974;50;780-787

LMCA from the RSV

Anand 2008

LMCA from the RSV

Basso C. JACC 2000; 35(6):1493-501

LMCA from the RSV

Basso C. JACC 2000; 35(6):1493-501

LCX from the RSV

ALCAPA

ALCAPA – CT Angio

Courtesy of Dr. Anne Marie Valente, Children’s Hospital Boston

ALCAPA - MRA

Courtesy of Dr. Anne Marie Valente, Children’s Hospital Boston

ALPACA - Echo

Courtesy of Dr. Anne Marie Valente, Children’s Hospital Boston

Agenda

• Anatomy and epidemiology

• Physiology and Risk Assessment

• Case studies

• Clinical Management

Clinical Management: ACC/AHA Guidelines

J. Am. Coll. Cardiol. 2008;52;e1-e121

Clinical Management – IVUS Study

Romp R. Ann Thorac Surg 2003;76:589-596

Unroofing procedure Osteoplasty

Surgical Approach

Clinical Management

• Medical therapy• Coronary ostial

stenting• Surgical repair

UnroofingOsteoplastyReimplantationCoronary bypass

graftingPicture courtesy of Dr. Fred Wu, Children’s Hospital Boston

Summary

• Definitions, epidemiology, and optimal diagnosis / management remains controversial and difficult to study

• Exclusion of anomalous coronaries critical in patients surviving SCD, or in younger patients with worrisome symptoms

• ~ 1-5% of angiograms; series anomalies rare but significant on a population scale

• Relatively large share of SCD in young patients• Combination of CTA / MRA / TTE / TEE / IVUS• Corrective repair recommended for LCA from RSV, any inter-arterial

lesion, and ALCAPA• Therapy for other lesions is unclear and typically tailored individually