title: Surgical management of the aortic root in patients with Marfan syndrome.
abstract: OBJECTIVE
  Surgical aortic root reconstruction techniques are standard therapy to avoid catastrophic vascular events in patients with Marfan syndrome with a dilated and/or dissected aortic root. The study aim was to evaluate the long-term results of aortic root reconstruction.
  
  
  METHODS
  Eighty-three patients (54 males, 29 females; mean age 37+/-17 years) fulfilling strict Ghent criteria for Marfan syndrome underwent aortic root surgery between 1971 and 2001. Of these patients, 65 (78%) underwent a composite valve conduit repair and 18 (22%) a valve-sparing aortic root reconstruction. Six patients (7%) suffered from an acute type A dissection, and 16 (19%) a chronic type A dissection.
  
  
  RESULTS
  In-hospital and 30-day mortality was 3.6% (n = 3). Morbidity included stroke (1.2%; n = 1), perioperative myocardial infarction (1.2%; n = 1) and reoperation for bleeding (10%; n = 8). Of 21 late deaths, the cause was cardiac in nine cases. Actuarial survival at 5, 10, 15 and 20 years was 84% (95% CI 76-93%), 73% (CI 61-86%), 59% (CI 45-77%) and 43% (CI 26-72%), respectively. Multivariate predictors for late death were postoperative dysrhythmias and need for inotropes (p < or =0.01). Freedom from reoperation at 5, 10, 15 and 20 years was 86% (CI 78-95%), 69% (CI 56-85%), 53% (CI 38-74%) and 48% (CI 23-71%), respectively. Multivariate predictors for reoperation were preoperative mitral valve prolapse and an initial valve-sparing aortic procedure (p < or =0.05). In the composite valve conduit patients, freedom from thromboembolism was 88% (CI 76-100%), and from endocarditis was 99% (CI 93-100%) at 15 years.
  
  
  CONCLUSIONS
  Composite valve conduit replacement of the aortic root in patients with Marfan syndrome offers a durable result, with low mortality and long-term complication rates. Reoperation was most commonly required for cardiac and vascular disease unrelated to the initial operation and in patients undergoing a valve-sparing aortic root procedure.
authors.affiliation: Division of Cardiovascular Surgery, Mayo Clinic, Rochester, MN 55905, USA. zehr.kenton@mayo.edu
authors.forename: Kenton J
authors.initials: KJ
authors.lastname: Zehr
authors.forename: Alireza
authors.initials: A
authors.lastname: Matloobi
authors.forename: Heidi M
authors.initials: HM
authors.lastname: Connolly
authors.forename: Thomas A
authors.initials: TA
authors.lastname: Orszulak
authors.forename: Francisco J
authors.initials: FJ
authors.lastname: Puga
authors.forename: Hartzell V
authors.initials: HV
authors.lastname: Schaff
mesh_terms: D000328:Adult; D000784:Aortic Dissection / Q000601:surgery*; D001014:Aortic Aneurysm / Q000601:surgery*; D001021:Aortic Valve / Q000601:surgery*; D001145:Arrhythmias, Cardiac / Q000150:complications; D002316:Cardiotonic Agents / Q000627:therapeutic use; D005260:Female; D005500:Follow-Up Studies; D006350:Heart Valve Prosthesis; D017052:Hospital Mortality; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000401:mortality / Q000601:surgery*; D008945:Mitral Valve Prolapse / Q000150:complications; D015999:Multivariate Analysis; D011183:Postoperative Complications; D012086:Reoperation / Q000706:statistics & numerical data; D012189:Retrospective Studies; D016019:Survival Analysis; D014184:Transplantation, Homologous
chemical_list: D002316:Cardiotonic Agents