Cardiovascular Events After Aortic Root Repair in Patients With Marfan Syndrome

J Am Coll Cardiol. 2023 Sep 12;82(11):1068-1076. doi: 10.1016/j.jacc.2023.06.032.


Background: The usefulness of aortic valve sparing operations to treat aortic root aneurysm in patients with Marfan syndrome (MS) remains controversial.

Objectives: The purpose of this study was to evaluate the occurrence of cardiovascular events in patients with MS who have undergone valve-preserving aortic root replacement.

Methods: Patients with MS who had aortic valve sparing operations (reimplantation of the aortic valve or remodeling of the aortic root) from 1988 through 2019 were followed prospectively for a median of 14 years. Pertinent data from clinical, echocardiographic, computed tomography, and magnetic resonance images of the aorta were collected and analyzed.

Results: There were 189 patients whose mean age was 36 years, and 67% were men. Ten patients presented with acute type A dissection and 29 had mitral regurgitation. There were 52 patients at risk at 20 years. Mortality rate at 20 years was 21.5% (95% CI: 14.7%-30.8%); advancing age and preoperative aortic dissections were associated with increased risk of death by multivariable analysis. At 20 years, the cumulative incidence of moderate or severe aortic insufficiency was 14.5% (95% CI: 9.5%-22.0%), reoperation on the aortic valve was 7.5% (95% CI: 3.9%-14.7%), and new distal aortic dissections was 19.9% (95% CI: 13.9%-28.5%). Remodeling of aortic root was associated with greater risk of developing aortic insufficiency and aortic valve reoperation than reimplantation of the aortic valve.

Conclusions: Aortic valve sparing operations provide stable aortic valve function and low rates of valve-related complications during the first 2 decades of follow-up but aortic dissections remain problematic in patients with MS.

Keywords: Marfan syndrome; aortic root aneurysm; aortic valve sparing operation; reimplantation of the aortic valve; remodeling of the aortic root.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aorta / diagnostic imaging
  • Aorta / surgery
  • Aorta, Thoracic
  • Aortic Dissection* / epidemiology
  • Aortic Dissection* / etiology
  • Aortic Dissection* / surgery
  • Aortic Valve Insufficiency* / epidemiology
  • Aortic Valve Insufficiency* / etiology
  • Aortic Valve Insufficiency* / surgery
  • Female
  • Humans
  • Male
  • Marfan Syndrome* / complications