Double-Lumen Aortic Arch: Persistence of the Fifth Aortic Arch

A persistent fifth aortic arch is exceptionally rare. We report a patient with a double-lumen aortic arch in association with a perimembranous ventricular septal defect and Cornelia de Lange syndrome. We also discuss the morphologic consequences of persistent fifth aortic, arch, which may not be limited to the double-lumen aorta but in fact may be more common.