Aortic valve degeneration after valve-sparing root replacement in patients with bicuspid aortopathy or connective tissue disorder

Objective: We sought to evaluate outcomes of valve-sparing root replacement (VSRR) in patients with bicuspid aortopathy (BAV) versus other connective tissue disorder (CTD). Methods: This was a single-center cohort study of consecutive patients undergoing VSRR via reimplantation from 2000 to 2023 with BAV or CTD. Operative outcomes, Kaplan-Meier survival estimates, and cumulative risk of reoperation and recurrent aortic insufficiency (AI) with the competing risk of death were assessed. Results: Of 516 patients who underwent VSRR, 109 (51.9%) had BAV and 101 (48.1%) had CTD. Patients with BAV were older (46.9 ± 10.4 vs 38.4 ± 14 years, P < .001) and more likely male (89.0% vs 56.4%, P < .001) and hypertensive (66.1% vs 28.7%, P < .001). Preoperative AI was similar (P = .57) between groups (30.3% mild, 18.3% moderate, 11.1% severe). Most patients had no/trivial immediate postoperative residual AI (96.3% vs 93.1%). Operative mortality was zero; postoperative adverse events were minimal. Mean clinical follow-up was 5.2 ± 4.4 years; 10-year survival was 95.6% versus 95.7% (P = .70). Echocardiographic follow-up was 3.9 ± 4.1 years; incidence of >2+ AI (9.7% vs 10.1%, P = 1.0) was similar between groups, whereas the incidence of moderate or greater aortic stenosis was greater with BAV (7.5% vs 0%, P = .02). Reoperation was low in both groups (3.7% vs 5.9%, P = .65). Competing risk analysis found no difference in reoperation hazard between BAV and CTD groups (hazard ratio, 0.36; 95% confidence interval, 0.07-1.81, P = .21). Conclusions: Patients with BAV and CTD have excellent operative outcomes, no mortality, and minimal residual AI after VSRR. Although the incidence of recurrent AI was similar, patients with BAV are at risk for AS.