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Abstract

Anomalous left coronary artery to pulmonary artery (ALCAPA) is a congenital heart defect in which the left coronary artery (LCA) originates from the pulmonary trunk instead of from the aorta. This disease occurs in 1 in 300,000 births and, if untreated, 90% of these neonates die within the first year. Individuals who live beyond this tend to be asymptomatic and either experience sudden death at an average age of 35 or present with cardiac abnormalities, including myocardial ischemia, arrhythmia, or mitral regurgitation. There are various surgical interventions used to treat ALCAPA, and the establishment of a two coronary artery system is the preferred treatment of choice. We report a case of a five-year-old presenting with supraventricular tachycardia since birth who, upon diagnosis of ALCAPA by angiography, was treated by surgical ligation. Thirty years later, he returned to a cardiologist with symptoms of congestive heart failure. The initial presentation of this patient is unusual because the manifestation of ALCAPA usually occurs within the first few months of birth and because ligation is no longer the preferred method of intervention. We discuss this unique case, suggest possible associations between ALCAPA and arrhythmias, review the various surgical methods used to treat ALCAPA, and evaluate the long-term outcome of ligation.