Anomalous Aortic Origin of a Coronary Artery

Anomalous aortic origin of a coronary artery (AAOCAAnomalous aortic origin of a coronary artery (AAOCA)), is a rare lesion whereby one or both coronary arteriesCoronary artery does not arise from the usual location on the aorta. It is the second leading cause of sudden cardiac deathSudden cardiac death in otherwise healthy young individuals. However, AAOCAAnomalous aortic origin of a coronary artery (AAOCA) remains a lesion with many unknowns. These relate to the true prevalence of this lesion, as most patients are incidentally diagnosed without any clinical consequences, and a lack of understanding regarding which anatomical variants are at high risk for ischemiaIschemia and sudden cardiac eventsSudden cardiac event. This leads to an unclear picture regarding the optimal management for patients, whether surgical or medical. In addition, while repair of AAOCAAnomalous aortic origin of a coronary artery (AAOCA) can be achieved using many techniques, the long-term outcomes of each strategy remains unknown, and recently the higher-than-expected complications of repair were outlined in a multi-institutional Congenital Heart Surgeons’ Society study. This chapter sheds light on these unknowns and provides clinicians with the current understandingsSudden cardiac arrest regarding how to best manage this complex lesion.