Aortic Valve-Sparing Root Operation in Patients With Marfan and Loeys-Dietz Syndrome.

Abstract

The objective of this study was to evaluate the midterm outcomes of valve-sparing aortic root replacement (VSRR) in patients with Marfan (MFS) and Loeys-Dietz syndrome (LDS). Additionally, the study analyzed operative factors, including the impact of graft selection. All patients with either MFS (158; 84%) or LDS (30; 16%) who underwent VSRR were included in a retrospective analysis from a transatlantic registry. Among 188 patients who had VSRR, 10 (5%) required reoperation on the aortic valve (7 with MFS and 3 with LDS; hazard ratio [HR] for LDS, 3.25; P = .078). Freedom from aortic valve reoperation at 10 years was 94% for patients with MFS and 87% for patients with LDS. A total of 21 patients (11%; 19 with MFS and 2 with LDS) experienced aortic valve regurgitation ≥2 (HR for LDS, 0.78; P = .755). One patient died 3 days after a severe stroke, and 7 patients died during follow-up (7 with MFS and 1 with LDS; HR LDS, 1.82; P = .595). During follow-up, 22 patients required an aortic reintervention (19 with MFS and 3 with LDS; P = .752). Patients with a Valsalva graft experienced significantly fewer total aortic reinterventions compared with patients with a straight graft (HR for Valsalva graft, 0.04; P < .001). VSRR shows excellent midterm outcomes in patients with MFS and LDS. There are no outcome differences between patients with MFS and patients with LDS. The use of a Valsalva graft may be considered to reduce aortic reintervention rates in patients with MFS and LDS.