Outcomes of Aortic Valve-Sparing Operations in Marfan Syndrome

J Am Coll Cardiol. 2015 Sep 29;66(13):1445-53. 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.

Keywords: aortic dissection; aortic root aneurysm; aortic insufficiency.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aortic Aneurysm / diagnosis
  • Aortic Aneurysm / mortality*
  • Aortic Aneurysm / surgery*
  • Aortic Valve / pathology
  • Aortic Valve / surgery
  • Child
  • Female
  • Follow-Up Studies
  • Heart Valve Prosthesis Implantation / mortality*
  • Heart Valve Prosthesis Implantation / trends
  • Humans
  • Male
  • Marfan Syndrome / diagnosis
  • Marfan Syndrome / mortality*
  • Marfan Syndrome / surgery*
  • Middle Aged
  • Prospective Studies
  • Retrospective Studies
  • Treatment Outcome
  • Young Adult