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Decision making in anomalous aortic origin of a coronary artery.

Anomalous aortic origin of a coronary artery (AAOCA) is the second most common cause of sudden cardiac death (SCD) in young athletes in United States. The exact pathophysiological mechanisms of SCD are unknown. There is lack of long-term outcome data on repaired and unrepaired AAOCA and our current risk stratification scheme for these patients is suboptimal. These patients are evaluated in a nonuniform manner across institutions in United States, and even by different providers residing in the same institution. The main objective of this article is to use what is known and unknown about this disease and to provide a possible framework that can help workup and manage patients with AAOCA in a more consistent fashion.