Total aortic replacement in Loeys-Dietz syndrome

Loeys-Dietz syndrome (LDS) is a recently identified genetic complex characterized in part by rapidly progressive aortic and branch vessel disease. We now describe total aortic replacement using an open Extent II thoracoabdominal repair followed by second-stage redo-sternotomy for a valve-sparing aortic root replacement and hybrid aortic arch repair in a patient with this syndrome.