title: Aortic root replacement in 372 Marfan patients: evolution of operative repair over 30 years.
abstract: BACKGROUND
  We reviewed the evolution of practice and late results of aortic root replacement (ARR) in Marfan syndrome patients at our institution.
  
  
  METHODS
  A retrospective clinical review of Marfan patients undergoing ARR at our institution was performed. Follow-up data were obtained from hospital and office records and from telephone contact with patients or their physicians.
  
  
  RESULTS
  Between September 1976 and September 2006, 372 Marfan syndrome patients underwent ARR: 269 had a Bentall composite graft, 85 had valve-sparing ARR, 16 had ARR with homografts, and 2 had ARR with porcine xenografts. In the first 24 years of the study, 85% received a Bentall graft; during the last 8 years, 61% had a valve-sparing procedure. There was no operative or hospital mortality among the 327 patients who underwent elective repair; there were 2 deaths among the 45 patients (4.4%) who underwent emergent or urgent operative repair. There were 74 late deaths (70 Bentalls, 2 homograft, and 2 valve-sparing ARRs). The most frequent causes of late death were dissection or rupture of the residual aorta (10 of 74) and arrhythmia (9 of 74). Of the 85 patients who had a valve-sparing procedure, 40 had a David II remodeling operation; there was 1 late death in this group, and 5 patients required late aortic valve replacement for aortic insufficiency. A David I reimplantation procedure using the De Paulis Valsalva graft has been used exclusively since May 2002. All 44 patients in this last group have 0 to 1+ aortic insufficiency.
  
  
  CONCLUSIONS
  Prophylactic surgical replacement of the ascending aorta in patients with Marfan syndrome has low operative risk and can prevent aortic catastrophe in most patients. Valve-sparing procedures, particularly using the reimplantation technique with the Valsalva graft, show promise but have not yet proven as durable as the Bentall.
authors.affiliation: Division of Cardiac Surgery, The Johns Hopkins Medical Institutions, Baltimore, Maryland 21287, USA. dcameron@jhmi.edu
authors.forename: Duke E
authors.initials: DE
authors.lastname: Cameron
authors.forename: Diane E
authors.initials: DE
authors.lastname: Alejo
authors.forename: Nishant D
authors.initials: ND
authors.lastname: Patel
authors.forename: Lois U
authors.initials: LU
authors.lastname: Nwakanma
authors.forename: Eric S
authors.initials: ES
authors.lastname: Weiss
authors.forename: Luca A
authors.initials: LA
authors.lastname: Vricella
authors.forename: Harry C
authors.initials: HC
authors.lastname: Dietz
authors.forename: Philip J
authors.initials: PJ
authors.lastname: Spevak
authors.forename: Jason A
authors.initials: JA
authors.lastname: Williams
authors.forename: Brian T
authors.initials: BT
authors.lastname: Bethea
authors.forename: Torin P
authors.initials: TP
authors.lastname: Fitton
authors.forename: Vincent L
authors.initials: VL
authors.lastname: Gott
mesh_terms: D000293:Adolescent; D000328:Adult; D017545:Aortic Aneurysm, Thoracic / Q000401:mortality / Q000601:surgery; D001021:Aortic Valve / Q000601:surgery*; D001022:Aortic Valve Insufficiency / Q000209:etiology* / Q000401:mortality / Q000601:surgery*; D001807:Blood Vessel Prosthesis / Q000009:adverse effects; D002423:Cause of Death; D005260:Female; D006350:Heart Valve Prosthesis / Q000009:adverse effects; D019918:Heart Valve Prosthesis Implantation / Q000009:adverse effects / Q000401:mortality; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000150:complications / Q000601:surgery*; D012092:Replantation / Q000009:adverse effects; D012189:Retrospective Studies; D015996:Survival Rate; D017741:Survivors; D014183:Transplantation, Heterologous; D014184:Transplantation, Homologous