To determine the experience with anomalous right coronary artery arising from the pulmonary artery (ARCAPA) at our institution.

Anomalous right coronary artery from the pulmonary artery is a rare congenital defect affecting 0.002% of the population. Unlike anomalous left coronary artery from the pulmonary artery (ALCAPA), ARCAPA generally has not been considered to be a lethal defect in infancy or childhood, although case reports of sudden death exist. The natural history of ARCAPA is difficult to describe because of the relatively small number of cases reported. Medical knowledge of this defect has been collected from case reports alone. No pediatric series of ARCAPA has been published.

Hospital databases were retrospectively searched for cases of ARCAPA diagnosed at the New York Presbyterian Hospital over the past 25 years. A comprehensive literature search for case reports of ARCAPA was performed.

Seven pediatric cases are described. The cases range in clinical presentation from a young infant with tetralogy of Fallot to an adolescent with chest pain on exertion. Only 1 patient came to medical attention because of chest pain; 6 patients were asymptomatic. In these 6, ARCAPA was diagnosed upon evaluation of either a murmur (3) or associated congenital heart disease (3). All 7 patients have undergone repair. One patient died after repair of complex tetralogy of Fallot/pulmonary atresia. One patient developed a thrombus in the dilated right coronary artery years after repair. The thrombus resolved with anticoagulation.

Anomalous right coronary artery from the pulmonary artery is an uncommon coronary anomaly that, unlike ALCAPA, rarely leads to sudden death. Surgical correction is low risk and should be considered for all patients.