The AAOCA is a rare birth defect in the heart that occurs when a coronary artery arises from the wrong location on the main blood vessel, the aorta.

In fact, AAOCA is the second leading cause of sudden cardiac death in children and adolescents in the United States behind hypertrophic cardiomyopathy.

[citation needed] Surgical intervention is indicated for coronary artery abnormalities in symptomatic patients with AAOCA (particularly with origin of the LCA from the right sinus), such as those with serious ventricular tachyarrhythmias or documented myocardial ischemia.

[citation needed] Asymptomatic patients with the right coronary artery arising from the left sinus are managed on a case-by-case basis.

[citation needed] In 2009, The Congenital Heart Surgeons' Society (CHSS) established a North American Registry in order to study a large multi-institutional cohort of patients with AAOCA.