Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) diagnosed in children and adolescents

BACKGROUND: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare but potentially fatal congenital coronary anomaly associated with early infant mortality and sudden adult death. By the development or lack of coronary collateral, it can be classified as infantile or adult type. However, even with the compensatory mechanism in adult patients, there is an estimated 80 to 90% incidence of sudden death at the mean age of 35 years. METHODS: We enrolled 9 patients with ALCAPA within the age group 5 to 16 years. RESULTS: Only one patient developed symptoms (apsychia), whereas other patients were asymptomatic, and there was no evident left ventricular dysfunction found in any of the cases. CONCLUSION: With the development of imaging techniques, asymptomatic adult-type ALCAPA patients could be identified and diagnosed in childhood or adolescence. As a potential cause of sudden death, ALCAPA should be surgically repaired soon after the diagnosis.