Repair in infancy of right aortic arch with aberrant left brachiocephalic artery in the setting of a variant of the hypoplastic left heart syndrome.

A right aortic arch, with a retroesophageal transverse arch and an aberrant left brachiocephalic artery, is a rare anomaly by itself. When combined with severe mitral stenosis and hypoplasia of the left ventricle, it is rarer still. We describe the diagnosis and treatment of this rare anomaly in an infant using a modification of the Norwood operation.