Current Management of Anomalous Aortic Origin of a Coronary Artery: A Pan-Canadian Survey

BACKGROUND: Anomalous aortic origin of a coronary artery (AAOCA) is a significant cause of sudden cardiac death (SCD) in children and adolescents. The natural history of AAOCA and the pathophysiology of AAOCA-related SCD are poorly understood. Therefore, the evaluation and management of AAOCA remain controversial. This survey-based study aims to report the current AAOCA management tendencies in Canada. METHODS: We built a 23-question survey on AAOCA. Questions pertained to patient presentation, investigations, morphology of the anomaly, management, and follow-up. We sent the survey to all the Canadian congenital cardiac surgeons, pediatric cardiologists, and adult congenital cardiologists. Data were anonymized and analysis was performed using descriptive statistics. RESULTS: According to our survey participants (N = 47), patient age (94%) and amount of physical activity (60%) are the most influential factors when deciding whether to offer surgical correction. Aborted SCD, exercise-induced syncope, typical chest pain, and left jaw or arm pain are the most important clinical presentations indicating surgery. The most commonly used preoperative investigations are rest echocardiography (75%), electrocardiogram (68%), and exercise stress test (62%). Most respondents favor the unroofing procedure (78%) for surgical correction. For nonsurgical candidates, most physicians choose competitive exercise restriction (64%). CONCLUSION: We found a divergence between current practices and expert consensus guidelines regarding the treatment of asymptomatic left AAOCA with high-risk features. Our survey also revealed a lack of consensus among clinicians regarding the management of asymptomatic patients, very young patients, and those with right-sided AAOCA. Evidence-based criteria derived from sufficiently powered studies remain to be established to standardize AAOCA treatment.