Congenital Coronary Anomalies
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This document summarizes a case presentation on a 40-year-old previously healthy woman who was found unresponsive at home. She was brought to the hospital where cardiac biomarkers were markedly elevated, indicating a non-ST-segment myocardial infarction (NSTEMI). Angiography revealed several congenital coronary artery anomalies including coronary artery fistulae and an anomalous left main coronary artery originating from the right sinus of Valsalva with an intra-atrial course. The presentation reviews various congenital coronary artery anomalies that can cause myocardial ischemia or sudden death including anomalous coronary artery origins, coronary artery fistulae, and coronary artery stenosis or atresia.
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Congenital Coronary Anomalies
- 1. Cardiothoracic / Cardiology Combined Confernce Michael G. Katz, MD Fellow in Cardiovascular Disease University of Rochester April 4, 2011
- 2. 2 Case - 2009 40 year old white woman Generally healthy Found unresponsive at home EMS called by husband Pt describes being found at home with a “pulseless arrest” • Details not available Evaluated at Newark Wayne Hospital
- 3. 3 Cardiac Biomarkers were markedly elevated Patient transferred to RGH for management of NSTEMI and cardiac catheterization
- 5. 5 Network of fistula: pLCx PA (common orifice) pLAD PA (common orifice) Anomolous LM from right sinus of Valsalva with intraatrial course (between aorta and PA)
- 7. 7 Left Coronary Caudal RAO LAD Diag LM OM LCx Diagonal Slide courtesy of Dr. Fred S. Ling, MD
- 8. 8 Left Coronary LAO Cranial LAD Diags LCx OM LM Slide courtesy of Dr. Fred S. Ling, MD
- 9. 9 Right Coronary LAO Cranial RPL AM or RV branch PDA Crux AM PDA Slide courtesy of Dr. Fred S. Ling, MD
- 10. 10 Right Coronary RAO PDA AM SAN AM PDA RCA Slide courtesy of Dr. Fred S. Ling, MD
- 11. Congenital Anomalies of Coronary Arteries 11
- 12. 12 Overall, infrequent (<1%) findings on coronary arteriograph
- 13. Anomalies that cause myocardial ischemia Coronary artery fistulae • About half of the patients with a coronary artery fistula are asymptomatic, but the other half develops HF, infective endocarditis, mycocardial ischemia, or rupture of an aneurysm. • Can arise from any of the coronary arteries, and drain into the RV, RA, pulmonary artery, LV, or superior vena cava. • Hemodynamic assessment is the best method to identify the left-to- right shunt resulting from these fistulae 13
- 14. LCA origin from the pulmonary artery • generally manifest as HF and myocardial ischemia in the first 4 months of life. However, about 25% of patients survive to adolescence or adulthood when they develop mitral regurgitation, angina, or HF. • Aortography demonstrates a large RCA with the absence of a left coronary ostium in the left aortic sinus. During the late phase of an aortogram, patulous LAD and LCx branches are seen filling by collateral circulation from RCA branches. Retrograde flow from the LAD and LCx opacifies the LMCA and its origin from the main pulmonary artery. The clinical course of the patient tends to be more favorable if extensive collateral circulation exists. 14
- 15. Congenital coronary stenosis or atresia of a coronary artery • an isolated lesion or in association with other congenital diseases (e.g., calcific coronary sclerosis, supravalvular aortic stenosis, homocystinuria, Friedreich's ataxia, Hurler's syndrome, progeria, and rubella syndrome). • When this occurs, the atretic vessel usually fills by means of collateral circulation from the contralateral side 15
- 16. Anomalous origin of either coronary artery from the contralateral sinus • Associated with sudden death during exercise in young persons. • Attributed to myocardial ischemia and electrical instability due to a slit- like, flow-limiting ostium with acute takeoff angles of the aberrant coronary arteries or by compression between the pulmonary trunk and aorta. • Origin of the RCA from the LCA or left aortic sinus with passage between the aorta and the RV outflow tract is somewhat less dangerous, but has also been associated with sudden death. 16
- 17. LM from the right: 4 possibilities “Dot and eye” technique More aptly: Dot -or- Eye technique 17 Anterior to PA Retro-aortic Intramyocardial (Septal) Intra-arterial (between Ao and PA)
- 18. 18 “Eye”
- 19. 19 “Dot”
- 20. 20 “Eye”
- 21. 21 “Dot”
- 23. “Dot and eye” 23
- 24. 24