Anomalous aortic origin of a coronary artery

Anomalous aortic origin of a coronary artery (AAOCA), from the inappropriate aortic arch with an interarterial, intraconal, or intramural course, is a rare heart defect associated with an increased risk of sudden death in children. Anomalous aortic origin of a coronary artery (AAOCA), from the inappropriate aortic arch with an interarterial, intraconal, or intramural course, is a rare heart defect associated with an increased risk of sudden death in children. 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.