Williams syndrome and obstructed total anomalous pulmonary venous return: a previously unreported association

Abstract

We report a case of a neonate who presented with obstructed mixed total anomalous pulmonary venous return (TAPVR) and Williams syndrome. After initial repair of TAPVR, his aortic wall continued to thicken, resulting in aortic arch hypoplasia and near atresia of the entire course of the left main coronary artery requiring a novel type of repair at 5 months of age. Cardiovascular abnormalities are frequently associated with Williams syndrome. This is a report of a rare patient with both Williams syndrome and obstructed TAPVR.