title: Valve-sparing aortic root replacement in patients with Marfan syndrome enrolled in the National Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions.
abstract: OBJECTIVE
  The long-term outcomes of aortic valve-sparing (AVS) root replacement in Marfan syndrome (MFS) patients remain uncertain. The study aim was to determine the utilization and outcomes of AVS root replacement in MFS patients enrolled in the Registry of Genetically Triggered Thoracic Aortic Aneurysms and Cardiovascular Conditions (GenTAC).
  
  
  METHODS
  At the time of this analysis, 788 patients with MFS were enrolled in the GenTAC Registry, of whom 288 had undergone aortic root replacement. Patients who had undergone AVS procedures were compared to those who had undergone aortic valve replacement (AVR).
  
  
  RESULTS
  AVS root replacement was performed in 43.5% of MFS patients, and the frequency of AVS was increased over the past five years. AVS patients were younger at the time of surgery (31.0 versus 36.3 years, p = 0.006) and more likely to have had elective rather than emergency surgery compared to AVR patients, in whom aortic valve dysfunction and aortic dissection was the more likely primary indication for surgery. After a mean follow up of 6.2 +/- 3.6 years, none of the 87 AVS patients had required reoperation; in contrast, after a mean follow up of 10.5 +/- 7.6 years, 11.5% of AVR patients required aortic root reoperation. Aortic valve function has been durable, with 95.8% of AVS patients having aortic insufficiency that was graded as mild or less.
  
  
  CONCLUSIONS
  AVS root replacement is performed commonly among the MFS population, and the durability of the aortic repair and aortic valve function have been excellent to date. These results justify a continued use of the procedure in an elective setting. The GenTAC Registry will be a useful resource to assess the long-term durability of AVS root replacement in the future.
authors.forename: Howard K
authors.initials: HK
authors.lastname: Song
authors.forename: Liliana R
authors.initials: LR
authors.lastname: Preiss
authors.forename: Cheryl L
authors.initials: CL
authors.lastname: Maslen
authors.forename: Barbara
authors.initials: B
authors.lastname: Kroner
authors.forename: Richard B
authors.initials: RB
authors.lastname: Devereux
authors.forename: Mary J
authors.initials: MJ
authors.lastname: Roman
authors.forename: Kathryn W
authors.initials: KW
authors.lastname: Holmes
authors.forename: H Eser
authors.initials: HE
authors.lastname: Tolunay
authors.forename: Patrice
authors.initials: P
authors.lastname: Desvigne-Nickens
authors.forename: Federico M
authors.initials: FM
authors.lastname: Asch
authors.forename: Rita K
authors.initials: RK
authors.lastname: Milewski
authors.forename: Joseph
authors.initials: J
authors.lastname: Bavaria
authors.forename: Scott A
authors.initials: SA
authors.lastname: LeMaire
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000784:Aortic Dissection / Q000209:etiology / Q000601:surgery; D001011:Aorta / Q000601:surgery*; D001014:Aortic Aneurysm / Q000209:etiology / Q000601:surgery; D001021:Aortic Valve / Q000601:surgery; D019917:Blood Vessel Prosthesis Implantation; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000150:complications / Q000601:surgery*; D008875:Middle Aged; D012042:Registries; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult