Outcomes of Aortic Valve-Sparing Operations in Marfan Syndrome

• Published in: Journal of the American College of Cardiology 2015-09, Vol.66 (13), p.1445-1453
• Main Authors: David, Tirone E., MD, David, Carolyn M., BN, Manlhiot, Cedric, BSc, Colman, Jack, MD, Crean, Andrew M., MD, Bradley, Timothy, MB, ChB
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Aged; Aneurysms; Aortic Aneurysm - diagnosis; Aortic Aneurysm - mortality; Aortic Aneurysm - surgery; aortic dissection; aortic root aneurysm; Aortic Valve - pathology; Aortic Valve - surgery; aortic insufficiency; Cardiology; Cardiovascular; Cardiovascular disease; Child; Coronary vessels; Dissection; Estimates; Family medical history; Female; Follow-Up Studies; Heart Valve Prosthesis Implantation - mortality; Heart Valve Prosthesis Implantation - trends; Humans; Internal Medicine; Male; Marfan syndrome; Marfan Syndrome - diagnosis; Marfan Syndrome - mortality; Marfan Syndrome - surgery; Medical imaging; Middle Aged; Mortality; Patients; Prospective Studies; Retrospective Studies; Sinuses; Studies; Tomography; Treatment Outcome; Values; Young Adult
• ISSN: 0735-1097; 1558-3597
• DOI: 10.1016/j.jacc.2015.07.041

Abstract Background In many cardiac units, aortic valve-sparing operations have become the preferred surgical procedure to treat aortic root aneurysm in patients with Marfan syndrome, based on relatively short-term outcomes. Objectives This study examined the long-term outcomes of aortic valve-sparing operations in patients with Marfan syndrome. Methods All patients with Marfan syndrome operated on for aortic root aneurysm from 1988 through 2012 were followed prospectively for a median of 10 years. Follow-up was 100% complete. Time-to-event analyses were calculated using the Kaplan-Meier method with log-rank test for comparisons. Results A total of 146 patients with Marfan syndrome had aortic valve-sparing operations. Reimplantation of the aortic valve was performed in 121 and remodeling of the aortic root was performed in 25 patients. Mean age was 35.7 ± 11.4 years and two-thirds were men. Nine patients had acute, 2 had chronic type A, and 3 had chronic type B aortic dissections before surgery. There were 1 operative and 6 late deaths, 5 caused by complications of dissections. Mortality rate at 15 years was 6.8 ± 2.9%, higher than the general population matched for age and sex. Five patients required reoperation on the aortic valve: 2 for endocarditis and 3 for aortic insufficiency. Three patients developed severe, 4 moderate, and 3 mild-to-moderate aortic insufficiency. Rate of aortic insufficiency at 15 years was 7.9 ± 3.3%, lower after reimplantation than remodeling. Nine patients developed new distal aortic dissections during follow-up. Rate of dissection at 15 years was 16.5 ± 3.4%. Conclusions Aortic valve-sparing operations in patients with Marfan syndrome were associated with low rates of valve-related complications in long-term follow-up. Residual and new aortic dissections were the leading cause of death.