title: Outcomes of Aortic Valve-Sparing Operations in Marfan Syndrome.
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.
  
  
  OBJECTIVE
  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.
authors.affiliation: Divisions of Cardiovascular Surgery and Cardiology, Peter Munk Cardiac Centre, Toronto General Hospital, University of Toronto, Toronto, Ontario, Canada. Electronic address: tirone.david@uhn.ca.
authors.forename: Tirone E
authors.initials: TE
authors.lastname: David
authors.affiliation: Divisions of Cardiovascular Surgery and Cardiology, Peter Munk Cardiac Centre, Toronto General Hospital, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Carolyn M
authors.initials: CM
authors.lastname: David
authors.affiliation: Divisions of Cardiovascular Surgery and Cardiology, Peter Munk Cardiac Centre, Toronto General Hospital, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Cedric
authors.initials: C
authors.lastname: Manlhiot
authors.affiliation: Divisions of Cardiovascular Surgery and Cardiology, Peter Munk Cardiac Centre, Toronto General Hospital, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Jack
authors.initials: J
authors.lastname: Colman
authors.affiliation: Divisions of Cardiovascular Surgery and Cardiology, Peter Munk Cardiac Centre, Toronto General Hospital, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Andrew M
authors.initials: AM
authors.lastname: Crean
authors.affiliation: Divisions of Cardiovascular Surgery and Cardiology, Peter Munk Cardiac Centre, Toronto General Hospital, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Timothy
authors.initials: T
authors.lastname: Bradley
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001014:Aortic Aneurysm / Q000175:diagnosis / Q000401:mortality* / Q000601:surgery*; D001021:Aortic Valve / Q000473:pathology / Q000601:surgery; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D019918:Heart Valve Prosthesis Implantation / Q000401:mortality* / Q000639:trends; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000175:diagnosis / Q000401:mortality* / Q000601:surgery*; D008875:Middle Aged; D011446:Prospective Studies; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult
keywords: aortic dissection; aortic root aneurysm; aortic insufficiency