Valve-Sparing Aortic Root Replacement in Loeys-Dietz Syndrome

BackgroundLoeys-Dietz syndrome (LDS) is a recently recognized aggressive aortic disorder characterized by root aneurysm, arterial tortuosity, hypertelorism, and bifid uvula or cleft palate. The results of prophylactic root replacement using valve-sparing procedures (valve-sparing root replacement [VSRR]) in patients with LDS is not known.MethodsWe reviewed all patients with clinical and genetic (transforming growth factor-β receptor mutations)...