Objective: The durability of reimplanted myxomatous aortic valves in root replacements for patients with connective tissue disorders (CTD) is unclear; therefore, we sought to evaluate the long-term resilience of these repairs.
Methods: From January 1980 to January 2020, 214 patients with CTD and 645 without CTD underwent primary, elective aortic valve reimplantation operations at Cleveland Clinic. The CTD cohort included 164 (77%) with Marfan, 23 (11%) with Loeys-Dietz, and 7 (3.3%) with Ehlers-Danlos CTD. We accounted for differing patient characteristics between the groups by propensity score matching to compare outcomes, yielding 96 matched pairs. Longitudinal echocardiographic measures were compared using nonlinear mixed effects models.
Results: In the CTD cohort, there were no operative mortalities (30-day or in-hospital), 1 (0.47%) stroke, and 1 (0.47%) early in-hospital reoperation for valve dysfunction. Ten-year prevalence of no aortic regurgitation was 86%, mild 11%, and moderate 3%. Ten-year freedom from reoperation was 97%. In propensity matched cohorts, there were no significant differences in in-hospital outcomes, longitudinal aortic regurgitation and mean gradient, risk of reoperation on the aortic valve, or risk of late death.
Conclusions: Aortic valve reimplantation is a durable operation in patients with CTD and root aneurysms. These patients do not experience early degeneration of their reimplanted aortic valves.
Keywords: Marfan syndrome; aortic root aneurysm; aortic root replacement; aortic valve reimplantation; connective tissue disorder.
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