Durability of Mitral Valve Repair in Adult Connective Tissue Disease

BACKGROUND: Connective tissue diseases (CTDs) are known risk factors for the development of mitral valve disease. Despite this risk, mitral valve repair (MVr) in patients with CTD remains underused. This study sought to investigate the survival and durability of MVr in adult patients with CTD. METHODS: All patients with suspected or confirmed Marfan, Loeys-Dietz, and Ehlers-Danlos syndromes who underwent MVr at a single institution between 2012 and 2023 were identified through database review. Survival of this cohort was compared with survival in patients with degenerative mitral regurgitation (MR) who underwent first-time isolated MVr during the same period. Primary end points included 30-day mortality, predicted intermediate-term survival evaluated with Kaplan-Meier analysis, recurrent moderate or severe MR, and rate of mitral reintervention. RESULTS: A total of 29 patients with CTD and 1757 patients with degenerative MR underwent MVr. Notably, 83% (24 of 29) of patients with CTD had less than trivial MR immediately after repair. No difference in 30-day survival was observed between the groups (CTD 96.6% vs degenerative MR 98.0%; P = .411). Similarly, there was no difference in intermediate-term survival (P = .586). Median echocardiographic follow-up time for patients with CTD was 3.5years (interquartile range, 1.1-6.1 years), and none required mitral valve reintervention or experienced recurrence of significant MR at the last known follow-up. CONCLUSIONS: MVr can safely be performed in patients with CTD, with good short- and intermediate-term survival, as well as excellent durability. MVr should be considered a standard of care in patients with connective tissue disease.