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 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?

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 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

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

21. pulmonarytrunk RL N LMCA inter-arterial Anatomic Variants pre-pulmonic retro-aortic 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. Basso C. JACC 2000; 35(6):1493-501 Intermittent Ischemia Pathophysiology of Sudden Death

25. Causes of Sudden Death in 387 Young Athletes Causeno. of athletespercent Hypertrophic Cardiomyopathy10226.4 Commotio cordis7719.9 Coronary artery anomalies5313.7 LV hypertrophy of indeterminate causation297.5 Myocarditis205.2 Ruptured aortic aneurysm (Marfan’s)123.1 ARVD112.8 Tunneled (bridged) coronary artery112.8 Aortic stenosis102.6 Premature atherosclerosis102.6 Dilated cardiomyopathy92.3 Long QT syndrome30.8 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

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

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

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

46. Clinical Management – IVUS Study

47. Romp R. Ann Thorac Surg 2003;76:589-596 Unroofing procedureOsteoplasty Surgical Approach

48. 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

49. 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