Anomalous Right Coronary Artery from the Left Sinus does Not Require Surgery in an Asymptomatic Child

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1 Anomalous Right Coronary Artery from the Left Sinus does Not Require Surgery in an Asymptomatic Child Frank A. Pigula, MD Senior Associate, Cardiac Surgery Children s Hospital Boston Associate Professor of Surgery Harvard Medical School

2 Prevalence, Symptomology, and Natural History Prevalence Overall incidence in children estimated to be 0.17% RCA from L sinus~ 0.1% LCA from R sinus~ 0.03% Incidence of sudden death~ 0.5:100,000 per year in US Symptoms Subjective vs objective Natural History Largely unknown

3 Sudden Death in Young Adults;A 25 year Review of Autopsies in Military Recruits. Eckart et al. Ann Intern Med 2004;141: ,300,000 Military recruits Deaths 64 Cardiac Deaths 86% related to exertion 21 left CAOS 0 right CAOS

4 Prevalence, Symptomology, and Natural History Prevalence Overall incidence in children estimated to be 0.17% RCA from L sinus~ 0.1% LCA from R sinus~ 0.03% Incidence of sudden death~ 0.5:100,000 per year in US Symptoms Subjective vs objective Natural History Largely unknown

5 Prevalence, Symptomology, and Natural History Overall incidence in children estimated to be 0.17% 6,300,000 x.0017= 10,700 RCA from L sinus~ 0.1% 6,300,000 x.001= 6,300 LCA from R sinus~ 0.03% 6,300,000 x.0003= 1,890 (21 / 1890= 1.1%) Eckhart, et al 21 LCAOS 0 RCAOS

6 % ALMCA 5 ARCA 0 Unpublished data Boston Children s Hospital

7 Chest Pain 406 patients 7-21 years 150 Exertional Chest Pain 100 echos 92 Exercise Stress Tests 5/406 Cardiac Etiolgies 2 pericarditis 3 arrhythmias Freidman et al. Pediatrics 2011;128:239-45

8 What about provocative testing? Very Infrequently done Why??

9 What about provocative testing? Very Infrequently done Why?? No one wants a dead patient in the office

10 What about provocative testing? Very Infrequently done Why?? No one wants a dead patient in the office It doesn t Help

11 Clinical Profile of Congenital Coronary Artery Anomalies with Origin from the Wrong Sinus Leading to Sudden Death in Young Competitive Athletes. Basso et al JACC 2000;35: athletes US and Italian National Registries 10 symptomatic 2 asymptomatic 15 asymptomatic 9 EKG all normal 27 Deaths 23 left CAOS 4 right CAOS 6 Exercise Stress Test All normal

12 Treatment Options Surgical Vs Medical Vs Interventional

13 Surgical Treatment Romp et al Ann Thorac Surg 2003;76: patients; 1 late Ross for AI Frommelt et al. JTCVS 2011; 142: patients; no significant complications reported Mainwaring et al. Ann Thorac Surg 2011;92: patients; 7 (14%) surgical complications Boston Children s Unpublished Data 42 patients; 6 (14%) surgical complications

14 Boston Children s Treatment Complications 2/42 (5%) Required early reoperation Resuspension of Intercoronary Commissure Interposition graft to elevate PA off the LMCA 1/42 Required ECMO for Coronary Spasm 1/42 Required Sternal Debridement for Infection 1/42 Required treatment for Corneal Abrasion 1/42 Persistent AV Node Reentry Tachycardia 6/42 (14%) Experienced Notable Complications

15 Medical and Interventional Treatment Association with sudden death, anomalous coronary origin and STRENUOUS exercise is very strong: avoid sports/strenuous activities Beta-Blockers Interventional procedures: proximal stenting How do we Determine Efficacy??

16 Inherent Flaws in population studies and small clinical series make blanket recommendations impossible Autopsy studies fail to diagnose an unknown number of afflicted individuals. Provocative testing and symptoms are untrustworthy There are no solid data to infer the need for surgery in asymptomatic patients with anomalous RCA from the left

17 Conclusion What to do with an asymptomatic patient who comes to you with anomalous coronary origin?

18 Conclusion What to do with an asymptomatic patient who comes to you with anomalous coronary origin? Precise imaging Ostial location Intramural

19 Conclusion What to do with an asymptomatic patient who comes to you with anomalous coronary origin? Precise imaging Ostial location Intramural L from the R Surgery

20 Conclusion What to do with an asymptomatic patient who comes to you with anomalous coronary origin? Precise imaging Ostial location Intramural L from the R Surgery R from the L Restriction of activity +/- medical management is a reasonable alternative