title: Early and long-term results of a valve-sparing operation for Marfan syndrome.
abstract: BACKGROUND
  We have previously described the experience, rationale, and development of a valve preserving technique, but its role in patients with Marfan syndrome has not previously been defined. Here, we attempt to determine the early and long-term results, timing, and determinants of outcome of this operation in patients with Marfan syndrome.
  
  
  RESULTS
  Since 1979, 82 patients (73.2% of all patients with Marfan syndrome undergoing resection of aneurysm of the ascending aorta) were operated on using this technique. Ages ranged from 2 to 69 years (mean, 33.9 years). In all, there were 4 early deaths (4.9%), 2 with acute dissection and 2 with chronic aneurysm operated on as emergencies. There were no early deaths in 67 patients operated on electively. Actuarial survival for patients operated for chronic aneurysm was 94.2%, 94.2%, and 94.2% at 1, 5, and 10 years, respectively; that for acute dissection was 72.7%, 63. 6%, and 63.6%; and that for chronic dissection was 100%, 85.7%, and 75.0%. The probability of needing reoperation was 5.7%, 17.3%, and 17.3% at 1, 5, and 10 years. There were no instances of infective endocarditis or thromboembolic complications except in 2 patients operated on early in the series who had cusp extension. At the end of the follow-up, trivial or no aortic regurgitation was demonstrated in 33.3%, mild in 45.6%, moderate in 21.1%, and severe in 0.
  
  
  CONCLUSIONS
  Valve-sparing operations are feasible in most patients with Marfan syndrome; they are applicable to patients with both dissection and chronic aneurysm. The early and long-term results are encouraging. Results are better in the absence of dissection, and prophylactic operation is warranted in some cases.
authors.affiliation: National Heart and Lung Institute at Imperial College of Science, Technology and Medicine, Middlesex, UK.
authors.forename: E J
authors.initials: EJ
authors.lastname: Birks
authors.forename: C
authors.initials: C
authors.lastname: Webb
authors.forename: A
authors.initials: A
authors.lastname: Child
authors.forename: R
authors.initials: R
authors.lastname: Radley-Smith
authors.forename: M H
authors.initials: MH
authors.lastname: Yacoub
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001011:Aorta / Q000601:surgery*; D001014:Aortic Aneurysm / Q000601:surgery*; D001021:Aortic Valve / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D008875:Middle Aged; D016019:Survival Analysis