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Coronary Anomalies

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  1. Coronary Anomalies Daniel Kramer December 17, 2008

  2. Inspiration I – RAO Caudal

  3. Inspiration I – RAO Cranial

  4. Inspiration I – LAO - RCA

  5. Inspiration I – LAO - LCX

  6. Inspiration II – RAO Caudal

  7. Inspiration II – LAO Caudal

  8. Inspiration II – F1 Hazing Shot

  9. Inspiration II

  10. Inspiration II – Aortic and PA Catheters

  11. Lingering Questions • What is normal? • What is the riskand mechanismof sudden cardiac death in these patients? • What modalities provide useful diagnostic or prognostic information? • What is the optimal management for various anatomical oddities?

  12. Agenda • Anatomy and epidemiology • Physiology and Risk Assessment • Case studies • Clinical Managment

  13. Normal Anatomy Grossman’s Cardiac Catheterization, Angiography, and Inervention 2006

  14. Echocardiography: Normal RCA and LMCA

  15. Echocardiography – Normal LCA

  16. Cardiac MRI – Coronary Sequence

  17. What is normal? Lack of consensus on definitions and diagnosis Anatomy vs physiology Clinical significance 1% Rule? Circulation 2007;115:1296-1305

  18. 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

  19. Possible pathways for ACAOS • Retrocardiac • Retroaortic • Preaortic / Inter-arterial • Intraseptal / Intramural • Prepulmonary • AL = antero-left • AR = antero-right • P = posterior Circulation 2007;115:1296-1305

  20. pre-pulmonic pulmonary trunk RCA inter-arterial R L normal N retro-aortic Anatomic Variants Cartoon courtesy of Dr. Fred Wu, Children’s Hospital Boston

  21. pulmonary trunk pre-pulmonic inter-arterial R L N retro-aortic LMCA Anatomic Variants Cartoon courtesy of Dr. Fred Wu, Children’s Hospital Boston

  22. Agenda • Anatomy and epidemiology • Physiology and Risk Assessment • Case studies • Clinical Managment

  23. Mechanisms and Classification Circulation 2007;115:1296-1305

  24. Pathophysiology of Sudden Death Intermittent Ischemia Basso C. JACC 2000; 35(6):1493-501

  25. Causes of Sudden Death in 387 Young Athletes Maron BJ. JAMA 1996; 276:199-204

  26. 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

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

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

  29. Agenda • Anatomy and epidemiology • Physiology and Risk Assessment • Case studies • Clinical Managment

  30. RCA from the LSV Courtesy of Dr. Anne Marie Valente, Children’s Hospital Boston

  31. RCA from the LSV Courtesy of Dr. Anne Marie Valente, Children’s Hospital Boston

  32. RCA from the LSV Courtesy of Dr. Anne Marie Valente, Children’s Hospital Boston

  33. LMCA from the RSV Circulation 1974;50;780-787

  34. LMCA from the RSV Circulation 1974;50;780-787

  35. LMCA from the RSV Anand 2008

  36. LMCA from the RSV Basso C. JACC 2000; 35(6):1493-501

  37. LMCA from the RSV Basso C. JACC 2000; 35(6):1493-501

  38. LCX from the RSV

  39. ALCAPA

  40. ALCAPA – CT Angio Courtesy of Dr. Anne Marie Valente, Children’s Hospital Boston

  41. ALCAPA - MRA Courtesy of Dr. Anne Marie Valente, Children’s Hospital Boston

  42. ALPACA - Echo Courtesy of Dr. Anne Marie Valente, Children’s Hospital Boston

  43. Agenda • Anatomy and epidemiology • Physiology and Risk Assessment • Case studies • Clinical Management

  44. Clinical Management: ACC/AHA Guidelines J. Am. Coll. Cardiol. 2008;52;e1-e121

  45. Clinical Management – IVUS Study

  46. Surgical Approach Unroofing procedure Osteoplasty Romp R. Ann Thorac Surg 2003;76:589-596

  47. Clinical Management • Medical therapy • Coronary ostial stenting • Surgical repair • Unroofing • Osteoplasty • Reimplantation • Coronary bypass grafting Picture courtesy of Dr. Fred Wu, Children’s Hospital Boston

  48. 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