title: Management of aortic disease in Marfan Syndrome: a decision analysis.
abstract: BACKGROUND
  Marfan syndrome is a relatively common heritable disorder of connective tissue that affects numerous organ systems, but the most severe complication is aortic aneurysm and dissection. A variety of medical and surgical approaches are available for managing the cardiovascular complications. Our objective was to compare elective composite graft surgery, elective valve-sparing surgery, and medical management for patients with both Marfan syndrome and thoracic aortic disease on the basis of life expectancy with differing diameters of the aortic root and rate of increase in the aortic root size.
  
  
  METHODS
  A Markov decision analysis model was constructed to compare the 2 surgical options with watchful waiting with medical therapy.
  
  
  RESULTS
  For our base-case analysis of a 20-year-old patient with Marfan syndrome and thoracic aortic aneurysm, the aortic valve-sparing option was preferred. It extended life expectancy to 73.8 years compared with the medical treatment option (71.4 years) and with the composite graft surgery (72.7 years). Our results show that there is a better outcome for a patient with an aortic root diameter between 3.0 and 3.5 cm with early prophylactic surgery than with deferred or emergency surgery. Medical treatment was preferred when the aortic root diameter was smaller than 3.0 cm.
  
  
  CONCLUSIONS
  Although long-term follow-up data are not yet available, it appears that advances in the technique of valve-sparing surgery have made it the preferred option to composite graft, primarily to avoid the complications of anticoagulation. Our study indicates that patients who have an aortic root diameter of larger than 3.0 cm should be considered for prophylactic aortic surgery.
authors.affiliation: Division of Rheumatology, Department of Medicine, University of Pennsylvania, Philadelphia 19104, USA.
authors.forename: Seo Young
authors.initials: SY
authors.lastname: Kim
authors.forename: Neil
authors.initials: N
authors.lastname: Martin
authors.forename: Elizabeth C
authors.initials: EC
authors.lastname: Hsia
authors.forename: Reed E
authors.initials: RE
authors.lastname: Pyeritz
authors.forename: Daniel A
authors.initials: DA
authors.lastname: Albert
mesh_terms: D000328:Adult; D017545:Aortic Aneurysm, Thoracic / Q000209:etiology* / Q000473:pathology / Q000628:therapy*; D001021:Aortic Valve / Q000601:surgery; D019917:Blood Vessel Prosthesis Implantation; D003661:Decision Support Techniques; D006801:Humans; D008017:Life Expectancy; D008382:Marfan Syndrome / Q000150:complications*; D008390:Markov Chains; D012680:Sensitivity and Specificity; D012720:Severity of Illness Index