Late adverse cardiovascular events after aortic valve-sparing operations in patients with Marfan syndrome.

Abstract

This study examines the very long-term outcomes of aortic valve-sparing operations for the treatment of aortic root aneurysm in patients with Marfan syndrome at a high-volume center. Among 215 patients with aortic root aneurysm associated with Marfan syndrome, 209 (97%) had aortic valve-sparing operations. Patients have been followed prospectively for a median of 14.6 years, with 26 patients remaining at risk at 25 years. All-cause mortality was estimated using the Kaplan-Meier survival method. Competing-risk models, along with the cumulative incidence function, were used to estimate adverse event rates. Cause-specific hazards regression models were used to evaluate time to adverse events outcomes. At the time of surgery, patients' median age was 34 years; 142 (68%) were men; 18 (8.6%) had acute or chronic aortic dissections; 67 (32%) had moderate or severe aortic insufficiency; and 37 (17.7%) had mitral insufficiency. Aortic valve reimplantation was performed in 183 patients, and aortic root remodeling was performed in 26 patients. Cumulative events at 25 years were 36.6% for death from any cause, 10.5% for reoperation on the aortic valve, 20% for moderate or severe aortic insufficiency, and 17.7% for the development of new distal aortic dissections. Aortic root remodeling was associated with an increased risk of aortic valve reoperation and postoperative aortic insufficiency by multivariate analyses. Creation of neoaortic sinuses in a cylindrical graft reduced the risk of postoperative dissections. Aortic valve reimplantation provides more stable aortic valve function than aortic root remodeling. Aortic dissections remain a serious problem in Marfan syndrome, and strategies to reduce their incidence are needed.