Long-term durability of valve-sparing root replacement in patients with and without connective tissue disease

The goal of this study was to evaluate the long-term outcomes of valve-sparing root replacement in patients with connective tissue disease (CTD) and compare them with patients without CTD who underwent valve-sparing root replacement for root aneurysm.

Of 487 patients, 380 (78%) did not have CTD and 107 (22%) had CTD; 97 (91%) with Marfan syndrome, 8 (7%) with Loeys-Dietz syndrome, and 2 (2%) with Vascular Ehlers-Danlos syndrome. Operative and long-term outcomes were compared.

The CTD group was younger (36 ± 14 years vs 53 ± 12 years; P < .001), had more women (41% vs 10%; P < .001) and had less hypertension (28% vs 78%; P < .001) and bicuspid aortic valve (8% vs 28%; P < .001). Other baseline characteristics did not differ between the groups. Overall operative mortality was nil (P = 1.000); the incidence of major postoperative complications was 1.2% (0.9% vs 1.3%; P = 1.000) and did not differ between groups. Residual mild aortic insufficiency (AI) was more frequent in the CTD group (9.3% vs 1.3%, P < .001) with no difference in moderate or greater AI. Ten-year survival was 97.3% (97.2% vs 97.4%; log-rank P = .801). Of the 15 patients with residual AI, 1 had none, 11 remained mild, 2 had moderate, and 1 had severe AI on follow-up. Ten-year freedom from moderate/severe AI was 89.6% (hazard ratio, 1.05; 95% CI, 0.8-1.37; P = .750) and 10-year freedom from valve reoperation was 94.9% (hazard ratio, 1.21; 95% CI, 0.43-3.39; P = .717).

The operative outcomes as well as long-term durability of valve-sparing root replacement is excellent in patients with or without CTD. Valve function and durability are not influenced by CTD.