Abstract
Anomalous aortic origin of coronary arteries (AAOCA) is a significant cause of sudden cardiac death (SCD) in children and adolescents. This is a rare congenital anomaly in which a coronary artery does not originate from its respective sinus of Valsalva, and follows an abnormal course. Because most AAOCA patients are likely asymptomatic, the prevalence of AAOCA remains unknown. When symptomatic, patients mostly present with angina, syncope or dyspnea on exertion. The natural history of AAOCA and the pathophysiology of AAOCA-related SCD are poorly understood, thus AAOCA evaluation and management remain controversial.
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