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. Children and young adults with these defects can die suddenly, especially during or just after exercise. In fact, AAOCA is the second leading cause of sudden cardiac death in children and adolescents in the United States behind hypertrophic cardiomyopathy. The prevalence is estimated at 0.1% to 0.3% of the general population. Neither the true risk of sudden death nor the best way to treat these patients is known with certainty. Because of the risk of sudden death, doctors face the pressure to "do something" but in the absence of long-term follow-up data, the risks and benefits of different management options are unconfirmed.
Anomalous aortic origin of a coronary artery
Cystic tumour of the atrioventricular nodal region
Anomalous left coronary artery from the pulmonary artery
Progressive cardiac conduction defect
Dorsal intercalated segment instability
Spontaneous coronary artery dissection
Stenosis of pulmonary artery
Congenital fiber type disproportion