Aortic valve-sparing root operation in patients with Marfan and Loeys-Dietz syndrome

The objective of this study was to evaluate the mid-term outcomes of valve-sparing aortic root replacement (VSRR) in patients with Marfan (MFS) and Loeys-Dietz syndrome (LDS). Additionally, we 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. Out of 188 patients with VSRR, ten (5%) required reoperation on the aortic valve (7 MFS and 3 LDS, HR for LDS: 3.25, p = 0.078). Freedom from aortic valve reoperation at 10 years was 94% for MFS and 87% for LDS. 21 patients (11%, 19 MFS and 2 LDS) developed aortic valve regurgitation ≥ 2 (HR LDS: 0.78, p = 0.755). One patient died three days after a severe stroke and 7 patients died during follow-up (7 MFS and 1 LDS, HR LDS: 1.82, p = 0.595). During follow-up, 22 patients required an aortic reintervention (19 MFS and 3 LDS, p = 0.752). Patients with a Valsalva graft experienced significantly less total aortic reinterventions compared to those with a straight graft (HR for Valsalva graft: 0.04, p < 0.001). VSRR shows excellent mid-term outcomes in patients with MFS and LDS. There are no outcome differences between patients with MFS compared to LDS. The use of a Valsalva graft may be considered to reduce aortic reintervention rates in patients with MFS and LDS.