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

Background 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.Methods All patients with either MFS (158, 84%) or LDS (30, 16%) who underwent VSRR were included in a retrospective analysis from a transatlantic registry.Results 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).Conclusions 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.

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Publication Details

Journal
Open Access CRIS of the University of Bern
Published
2025-08-14
DOI
https://doi.org/10.48620/90669
Primary Topic
Connective tissue disorders research
Type
article
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article

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

Open Access CRIS of the University of Bern
Connective tissue disorders research
article

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

article en

Abstract

Background 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.Methods All patients with either MFS (158, 84%) or LDS (30, 16%) who underwent VSRR were included in a retrospective analysis from a transatlantic registry.Results 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).Conclusions 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.

Open Access CRIS of the University of Bern
United Heart and Vascular Clinic (US)
Openalex Percentile: Top 54%
Connective tissue disorders research
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