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Bentall vs. Valve-Sparing Procedures in Marfan Syndrome
Study Questions:
Among patients with Marfan syndrome who underwent surgery for aortic root aneurysm, what were the long-term outcomes associated with valve-sparing aortic root replacement (VSRR) compared with a Bentall procedure?
Methods:
Adult patients with Marfan syndrome who underwent Bentall or aortic VSRR procedures between 1997 and 2013 were identified. Comprehensive follow-up information was obtained from hospital charts and telephone contact.
Results:
A single-center cohort of 165 adult patients with Marfan syndrome (ages >20 years) underwent either VSRR (n = 98; 69 re-implantation, 29 remodeling) or Bentall (n = 67) procedures. Patients undergoing the Bentall procedure were older (median age 37 vs. 36 years; p = 0.03), had larger median preoperative sinus diameter (5.5 cm vs. 5.0 cm; p = 0.003), more aortic dissections (25.4% vs. 4.1%; p < 0.001), higher incidence of moderate or severe aortic insufficiency (49.3% vs. 14.4%; p < 0.001), and more urgent or emergent operations (24.6% vs. 3.3%; p < 0.001). There were no hospital deaths and nine late deaths in more than 17 years of follow-up (median follow-up 7.8 years). Ten-year survival was 90.5% in patients undergoing the Bentall procedure and 96.3% in patients undergoing VSRR (p = 0.10). Multivariable analysis revealed that VSRR was associated with fewer thromboembolic or hemorrhagic events (hazard ratio, 0.16; 95% confidence interval, 0.03-0.85; p = 0.03). There was no independent difference in long-term survival, freedom from reoperation, or freedom from endocarditis between the two procedures.
Conclusions:
After prophylactic root replacement in patients with Marfan syndrome, patients undergoing Bentall and valve-sparing procedures have similar late survival, freedom from root re-operation, and freedom from endocarditis. However, valve-sparing procedures result in significantly fewer thromboembolic and hemorrhagic events.
Perspective:
Prophylactic aortic root replacement improves survival in patients with Marfan syndrome with aortic root aneurysm, but the optimal procedure has not been well defined. These data suggest that, in the right hands, the more technically challenging valve-sparing root repair procedure has associated long-term clinical advantages in terms of fewer thromboembolic and hemorrhagic events; albeit with similar rates as a Bentall procedure in terms of late survival, freedom from root re-operation, and freedom from endocarditis. These data support the use of valve-sparing procedures when possible in order to avoid anticoagulation-related complications associated with mechanical valves. The Marfan population is a high-risk and specialized population, and referral to a reference center for intervention for aortic root aneurysm appears appropriate.
Keywords: Aneurysm, Dissecting, Aortic Aneurysm, Aortic Valve Insufficiency, Cardiac Surgical Procedures, Endocarditis, Heart Defects, Congenital, Heart Valve Diseases, Heart Valve Prosthesis, Hemorrhage, Marfan Syndrome, Reoperation, Survival, Thromboembolism
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