title: Long-term outcomes of aortic root operations for Marfan syndrome: A comparison of Bentall versus aortic valve-sparing procedures.
abstract: OBJECTIVE
  Prophylactic aortic root replacement improves survival in patients with Marfan syndrome with aortic root aneurysms, but the optimal procedure remains undefined.
  
  
  METHODS
  Adult patients with Marfan syndrome who had Bentall or aortic valve-sparing root replacement (VSRR) procedures between 1997 and 2013 were identified. Comprehensive follow-up information was obtained from hospital charts and telephone contact.
  
  
  RESULTS
  One hundred sixty-five adult patients with Marfan syndrome (aged > 20 years) had either VSRR (n = 98; 69 reimplantation, 29 remodeling) or Bentall (n = 67) procedures. Patients undergoing Bentall procedure were older (median, 37 vs 36 years; P = .03), had larger median preoperative sinus diameter (5.5 cm vs 5.0 cm; P = .003), more aortic dissections (25.4% vs 4.1%; P < .001), higher incidence of moderate or severe aortic insufficiency (49.3% vs 14.4%; P < .001) and more urgent or emergent operations (24.6% vs 3.3%; P < .001). There were no hospital deaths and 9 late deaths in more than 17 years of follow-up (median, 7.8 deaths). Ten-year survival was 90.5% in patients undergoing Bentall procedure and 96.3% in patients undergoing VSRR (P = .10). Multivariable analysis revealed that VSRR was associated with fewer thromboembolic or hemorrhagic events (hazard ratio, 0.16; 95% confidence interval, 0.03-0.85; P = .03). There was no independent difference in long-term survival, freedom from reoperation, or freedom from endocarditis between the 2 procedures.
  
  
  CONCLUSIONS
  After prophylactic root replacement in patients with Marfan syndrome, patients undergoing Bentall and valve-sparing procedures have similar late survival, freedom from root reoperation, and freedom from endocarditis. However, valve-sparing procedures result in significantly fewer thromboembolic and hemorrhagic events.
authors.affiliation: Division of Cardiac Surgery, The Johns Hopkins University School of Medicine, Baltimore, Md. Electronic address: jprice@jhmi.edu.
authors.forename: Joel
authors.initials: J
authors.lastname: Price
authors.affiliation: Division of Cardiac Surgery, The Johns Hopkins University School of Medicine, Baltimore, Md.
authors.forename: J Trent
authors.initials: JT
authors.lastname: Magruder
authors.affiliation: Division of Cardiac Surgery, The Johns Hopkins University School of Medicine, Baltimore, Md.
authors.forename: Allen
authors.initials: A
authors.lastname: Young
authors.affiliation: Division of Cardiac Surgery, The Johns Hopkins University School of Medicine, Baltimore, Md.
authors.forename: Joshua C
authors.initials: JC
authors.lastname: Grimm
authors.affiliation: Division of Cardiac Surgery, The Johns Hopkins University School of Medicine, Baltimore, Md.
authors.forename: Nishant D
authors.initials: ND
authors.lastname: Patel
authors.affiliation: Division of Cardiac Surgery, The Johns Hopkins University School of Medicine, Baltimore, Md.
authors.forename: Diane
authors.initials: D
authors.lastname: Alejo
authors.affiliation: Departments of Pediatrics, Medicine, Molecular Biology, and Genetics, The Johns Hopkins University School of Medicine, Baltimore, Md.
authors.forename: Harry C
authors.initials: HC
authors.lastname: Dietz
authors.affiliation: Division of Cardiac Surgery, The Johns Hopkins University School of Medicine, Baltimore, Md.
authors.forename: Luca A
authors.initials: LA
authors.lastname: Vricella
authors.affiliation: Division of Cardiac Surgery, The Johns Hopkins University School of Medicine, Baltimore, Md.
authors.forename: Duke E
authors.initials: DE
authors.lastname: Cameron
mesh_terms: D000328:Adult; D001011:Aorta / Q000473:pathology / Q000601:surgery*; D001014:Aortic Aneurysm / Q000175:diagnosis / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D001021:Aortic Valve / Q000473:pathology / Q000601:surgery*; D019917:Blood Vessel Prosthesis Implantation / Q000009:adverse effects / Q000379:methods* / Q000401:mortality; D004108:Dilatation, Pathologic; D018572:Disease-Free Survival; D005260:Female; D019918:Heart Valve Prosthesis Implantation / Q000009:adverse effects / Q000379:methods* / Q000401:mortality; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008382:Marfan Syndrome / Q000150:complications* / Q000175:diagnosis / Q000401:mortality; D008875:Middle Aged; D016016:Proportional Hazards Models; D012086:Reoperation; D012092:Replantation; D012189:Retrospective Studies; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome
keywords: Marfan syndrome; aortic root replacement; aortic valve-sparing root replacement