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Coronary Anomalies
Daniel Kramer
December 17, 2008
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Inspiration I – RAO Caudal
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Inspiration I – RAO Cranial
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Inspiration I – LAO - RCA
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Inspiration I – LAO - LCX
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Inspiration II – RAO Caudal
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Inspiration II – LAO Caudal
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Inspiration II – F1 Hazing Shot
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Inspiration II
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Inspiration II – Aortic and PA Catheters
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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?
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Agenda
• Anatomy and epidemiology
• Physiology and Risk Assessment
• Case studies
• Clinical Managment
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Normal Anatomy
Grossman’s Cardiac Catheterization, Angiography, and Inervention 2006
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Echocardiography: Normal RCA and LMCA
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Echocardiography – Normal LCA
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Cardiac MRI – Coronary Sequence
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What is normal?
Lack of consensus on definitions and diagnosis
Anatomy vs physiology
Clinical significance
1% Rule?
Circulation 2007;115:1296-1305
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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
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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
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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
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pulmonarypulmonarytrunktrunk
RR LL
NNLMCALMCA
inter-arterialinter-arterial
Anatomic Variants
pre-pulmonic
retro-aortic
Cartoon courtesy of Dr. Fred Wu, Children’s Hospital Boston
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Agenda
• Anatomy and epidemiology
• Physiology and Risk Assessment
• Case studies
• Clinical Managment
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Mechanisms and Classification
Circulation 2007;115:1296-1305
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Basso C. JACC 2000; 35(6):1493-501
Intermittent Ischemia
Pathophysiology of Sudden Death
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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
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• 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
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Basso C. JACC 2000; 35(6):1493-501
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Basso C. JACC 2000; 35(6):1493-501
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Agenda
• Anatomy and epidemiology
• Physiology and Risk Assessment
• Case studies
• Clinical Managment
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RCA from the LSV
Courtesy of Dr. Anne Marie Valente, Children’s Hospital Boston
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RCA from the LSV
Courtesy of Dr. Anne Marie Valente, Children’s Hospital Boston
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RCA from the LSV
Courtesy of Dr. Anne Marie Valente, Children’s Hospital Boston
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LMCA from the RSV
Circulation 1974;50;780-787
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LMCA from the RSV
Circulation 1974;50;780-787
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LMCA from the RSV
Anand 2008
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LMCA from the RSV
Basso C. JACC 2000; 35(6):1493-501
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LMCA from the RSV
Basso C. JACC 2000; 35(6):1493-501
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LCX from the RSV
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ALCAPA – CT Angio
Courtesy of Dr. Anne Marie Valente, Children’s Hospital Boston
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ALCAPA - MRA
Courtesy of Dr. Anne Marie Valente, Children’s Hospital Boston
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ALPACA - Echo
Courtesy of Dr. Anne Marie Valente, Children’s Hospital Boston
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Agenda
• Anatomy and epidemiology
• Physiology and Risk Assessment
• Case studies
• Clinical Management
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Clinical Management: ACC/AHA Guidelines
J. Am. Coll. Cardiol. 2008;52;e1-e121
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Clinical Management – IVUS Study
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Romp R. Ann Thorac Surg 2003;76:589-596
Unroofing procedure Osteoplasty
Surgical Approach
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Clinical Management
• Medical therapy• Coronary ostial
stenting• Surgical repair
UnroofingOsteoplastyReimplantationCoronary bypass
graftingPicture courtesy of Dr. Fred Wu, Children’s Hospital Boston
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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