title: Aortic surgery in patients with marfan syndrome: long-term survival, morbidity and function.
abstract: OBJECTIVE
  The natural history of patients with Marfan syndrome is depressing, but surgical intervention on the aorta can improve the prognosis. Study results were analyzed with reference to long-term survival, morbidity and function.
  
  
  METHODS
  Seventy-four Marfan patients (51 males, 23 females; mean age 41+/-14 years), underwent first-time aortic surgery between 1977 and 1998. Follow up information regarding mortality, morbidity and functional status was obtained from patient records and by questionnaire. The mean follow up was 5 years (range: 0-19 years). Forty-seven patients (64%) had a dissection, 27 (36%) an aneurysm, and 45 (61%) patients underwent emergency operations (<4 h from arrival at hospital). In 72 patients (97%) the disease affected the ascending aorta, and implantation of a composite graft was the most frequent operation.
  
  
  RESULTS
  Overall 30-day mortality was 12% (3% in elective cases, 18% in emergency cases, p <0.05). Emergency operations and surgery extended to the aortic arch were risk factors for early mortality. Overall actuarial survival was 63.4+/-8% at 10 years. Age was the only risk factor for late mortality. Seventeen patients were reoperated on due to pathologies of the remaining aorta (n = 12), pseudoaneurysms (n = 4) and aortic valve endocarditis (n = 1). A total of five patients had endocarditis; one patient with a homograft required surgery, and medical treatment was successful in the other four patients. Five patients had neurological thromboembolic episodes without permanent damage, and six had minor bleeding complications. Freedom from early and late mortality, reoperation on the aorta and major cardiovascular events (endocarditis episodes, thromboembolic/hemorrhagic strokes and other major bleeding/embolic episodes) was 33.3 +/- 8.1% at 10 years. At follow up, 98% of patients were in NYHA functional class I or II, and 80% were working.
  
  
  CONCLUSIONS
  Elective aortic surgery in Marfan patients can be performed with good results. Close follow up of patients undergoing surgery is important. The long-term functional status of surviving patients is satisfactory.
authors.affiliation: Department of Cardiothoracic Surgery, Sahlgrenska Uinversity Hospital, Gothenburg, Sweden.
authors.forename: V
authors.initials: V
authors.lastname: Lepore
authors.forename: A
authors.initials: A
authors.lastname: Jeppsson
authors.forename: G
authors.initials: G
authors.lastname: Rådberg
authors.forename: V
authors.initials: V
authors.lastname: Mantovani
authors.forename: M
authors.initials: M
authors.lastname: Bugge
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000784:Aortic Dissection / Q000235:genetics / Q000401:mortality / Q000601:surgery*; D017545:Aortic Aneurysm, Thoracic / Q000235:genetics / Q000401:mortality / Q000601:surgery*; D001021:Aortic Valve / Q000601:surgery; D001705:Bioprosthesis; D019917:Blood Vessel Prosthesis Implantation; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D005820:Genetic Testing; D019918:Heart Valve Prosthesis Implantation; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000235:genetics / Q000401:mortality / Q000601:surgery*; D008875:Middle Aged; D012086:Reoperation; D015996:Survival Rate