Anomalous left coronary artery, ventricular septal defect, and double aortic arch

Anomalous origin of the left coronary artery from the pulmonary artery generally occurs in isolation. We report an infant diagnosed with double aortic arch and ventricular septal defect who was found to have an anomalous origin of the left coronary artery from the pulmonary artery at surgery. Successful ventricular septal defect repair, division of the arch, and aortic reimplantation of the left coronary artery was performed. This article describes a combination of lesions previously not described, highlights the altered presentation when multiple lesions coexist, discusses the propensity to miss the anomalous coronary, and reviews the literature that demonstrates the consequences of overlooking this defect.