PM1008: A Survey on the Management of Anomalous Aortic Origins of the Coronary Arteries

Background: Anomalous aortic origins of the coronary arteries (AAOCA) can be associated with sudden cardiac death, but are most often identified incidentally. The management of patients with AAOCA remains controversial due to limitations in risk stratification and limited data on follow-up of surgical treatments. Despite the 2017 American Association for Thoracic Surgery (AATS) expert guidelines, providers face uncertainty when formulating treatment plans for these patients.

Methods: We sent out an anonymous electronic survey to the American Academy of Pediatrics Section on Cardiology and Cardiac Surgery (AAP SOCCS) and Pediheart.net online community regarding their practice patterns in the care of patients with AAOCA. The survey was limited to anomalous origins of the right coronary from the left cusp (AAORCA) and left coronary from the right cusp (AAOLCA) with inter-arterial courses.

Results: We received 103 complete responses. There were 98 pediatric cardiologists, of which 90 routinely read echocardiograms. Of those who had completed training, the mean years in practice was 15.9 years.

Respondents felt that the coronaries were adequately imaged in first-time echocardiograms at the following ages 84% ( < 1 year), 79% (1-4 years), 80% (5-9 years) and 72% (10-20 years). Across the age spectrum, less than ¼ of respondents recommended repeat echocardiography if the coronaries were not adequately visualized.

After incidental diagnosis of AAOCA at birth, the next cardiology visit, next echocardiogram and coronary CT were recommended at 1.50 (+/- 1.59), 1.93 (+/-2.09) and 5.69 (+/-3.89) years for AAOLCA and 1.92(+/-1.94), 2.35 (+/-2.23) and 6.08 (+/-3.89) years for AAORCA.

For a 16-year-old with an AAOCA and either symptoms of ischemia, no symptoms of ischemia but evidence of ischemia on stress imaging or no symptoms and no evidence of ischemia on stress imaging, in 5/6 vignettes, responses closely matched the AATS recommendations. However, in asymptomatic AAOLCA with no ischemia on stress imaging, surgery was felt to be appropriate or somewhat appropriate by 38.8% and 30.1% of respondents, with 31.1% of respondents giving less support for surgery despite the AATS guidelines recommending that surgery be offered.

In a 16-year-old with AAORCA with no evidence of ischemia on stress testing, respondents were more likely to recommend surgery in competitive athletes 43.7% and patients with hypertension 13.6% but less like to recommend it in patients with Down’s syndrome 13.6% and pulmonary hypertension 13.6%.

Conclusion: Our survey demonstrates that although the coronary arteries are generally well identified on routine echocardiography, there is a wide range in the practice management of AAOCA which may not correlate with previously published guidelines. These results may be useful when considering future guidelines for the management of AAOCA.