Outcomes of aortic valve-sparing root replacement with cusp repair in connective tissue disease.

Background: Although, valve sparing is commonly performed in patients with Marfan syndrome, feasibility and results of cusp repair for aortic insufficiency have not been studied.

Aim: To report on the outcomes and durability of aortic cusp repair in valve sparing in patients with Marfan syndrome.

Methods: All consecutive adult patients with Marfan syndrome who underwent remodelling and annuloplasty with aortic valve repair for aortic insufficiency between May 2005 and December 2020 were included. Patients with Marfan syndrome treated for aortic aneurysm, but without aortic insufficiency, were excluded. Data were collected prospectively and reviewed retrospectively from the Aorticvalve repair International Registry (AVIATOR).

Results: During the study period, 71 patients with Marfan syndrome were referred to surgery. Fifty-five patients with connective tissue disease and aortic insufficiency with aorta aneurysm were treated: 46 underwent aortic valve repair and nine underwent aortic valve replacement (five mechanical aortic valve replacements and four biological aortic valve replacements). The mean age was 42.9±15.4 years, and the mean EuroScore II was 2.5±2.2. No patient died, and no patient had significant aortic insufficiency (grade≥II) at discharge. The 5-year survival rate estimate was 94.4%, which seems statistically similar to that of the age- and sex-matched general population. At 5 years, freedom from reoperation was 94.6%, and the incidence of infective endocarditis was 2.6%. No valve thrombosis, aortic dissection, major bleeding events, thromboembolic events (stroke) or myocardial infarctions were noted during follow-up.

Conclusion: Remodelling and aortic valve repair showed excellent durability at 5 years, even in connective tissue disorders.