ALCAPA in an adult with preserved left ventricular function.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is the most common congenital coronary artery defect. Left uncorrected, nearly 90% of patients will die within one year. Without surgical repair, surviving patients are subjected to risks of myocardial ischemia with global cardiomyopathy, chronic mitral regurgitation, and sudden death. We report a case of ALCAPA in a 23-year-old female with completely preserved left ventricular function. This article reviews the mechanism of this unusual presentation as well as the therapeutic options for adults presenting with ALCAPA.