Abstract
Loeys-Dietz syndrome and vascular Ehlers-Danlos syndrome are genetic aortopathies that result from abnormal collagen matrix formation associated with vascular complications and early death. Identification of simultaneous COL3A1 and SMAD3 mutations as well as subsequent open and endovascular repair have not been reported. We present a case of a staged complete aortic replacement in a patient with a 7-cm aneurysm of his aortic arch and confirmed genetic mutations for Loeys-Dietz syndrome and vascular Ehlers-Danlos syndrome. This case highlights that, despite increased operative risk, successful staged repair of the entire aorta can be achieved in a patient with multiple severe genetic aortopathies.