Anomalous Aortic Origin of a Coronary Artery with an Interarterial Course: Understanding Current Management Strategies in Children and Young Adults

Anomalous aortic origin of a coronary artery from the opposite sinus of Valsalva with an interarterial course (AAOCA) is a rare congenital anomaly that is the second leading cardiovascular cause of sudden death in children and adolescents. Management practices with respect to this lesion vary among clinicians. We surveyed practitioners’ opinions on treatment and management strategies for patients with AAOCA. A 17-question “Patient Management Questionnaire” was sent to all Congenital Heart Surgeons’ Society (CHSS) members (n = 90), who were asked to respond and to distribute the questionnaire to pediatric cardiologists and nurse practitioners at their institutions. Questions addressed patient presentation, diagnostic modalities, therapeutic intervention, complications to therapy, follow-up visits, and type and frequency of follow-up testing. Descriptive statistics were used for analyses. We received 113 responses. Almost all respondents agree that ischemia at presentation or with provocative testing warrants surgical repair, whereas the majority believes that any patient with symptoms but no evidence of ischemia should have surgery. Approximately one-half and one-third of the respondents manage patients who are awaiting surgery or not having surgery by completely restricting exercise or not allowing competitive athletics, respectively. In asymptomatic patients, patient age determines timing of surgical repair but almost one-quarter did not believe that an asymptomatic child should undergo surgery. Among patients managed medically, there were six sudden deaths; there were two deaths in those who had surgical repair. There is marked heterogeneity in physician opinions regarding AAOCA treatment and management. A multicenter registry would facilitate the development of evidence-based treatment and management guidelines for children and young adults with AAOCA.