title: Midterm Survival and Quality of Life After Extent II Thoracoabdominal Aortic Repair in Marfan Syndrome.
abstract: BACKGROUND
  Pathologic conditions of the aorta remain a major source of morbidity and mortality for patients with Marfan syndrome (MFS). Extensive thoracoabdominal aortic aneurysm (TAAA) repair can prevent aortic catastrophe but carries substantial risk of perioperative adverse events. We evaluated midterm survival and quality of life (QoL) after contemporary Crawford extent II TAAA repair in patients with MFS.
  
  
  METHODS
  From 2004 to 2010, 49 consecutive patients with MFS (mean age, 43.4 ± 12.0 years) underwent extent II TAAA repair (41 elective and 8 urgent/emergent procedures) with intercostal reimplantation. Thirty-six patients (73%) had aorta-related symptoms, and 45 (92%) had distal aortic dissection. Operative adjuncts included cerebrospinal fluid drainage (n = 47 [96%]), left heart bypass (n = 46 [94%]), and cold renal perfusion (n = 47 [96%]). Kaplan-Meier survival analysis was performed. QoL was assessed in 24 patients with a 12-item survey (12-Item Short Form Health Survey version 2 [SF-12v2]) a median of 5.3 (interquartile range [IQR], 4.0-7.9) years postoperatively. QoL data were normalized and compared with data from the general population.
  
  
  RESULTS
  There were no operative deaths, strokes, paraparesis, or paraplegia. Two patients (4%) had permanent renal failure necessitating hemodialysis. The most frequent complication was vocal cord paralysis (n = 21 [43%]). Six-year Kaplan-Meier survival was 84% ± 6%. The 24 patients with QoL data had slightly worse physical component scores (46.0 ± 10.6) and slightly better mental component scores (51.4 ± 10.4) than the general population (50 ± 10 for both scores).
  
  
  CONCLUSIONS
  Operative treatment of extensive TAAA in patients with MFS enables excellent midterm survival and QoL. Cerebrospinal fluid drainage, left heart bypass, and cold renal perfusion probably aid in achieving excellent outcomes.
authors.affiliation: Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas; Department of Cardiovascular Surgery, Texas Heart Institute, Houston, Texas; CHI St. Luke's Health-Baylor St. Luke's Medical Center, Houston, Texas; Division of Thoracic and Cardiovascular Surgery, University of Virginia, Charlottesville, Virginia.
authors.forename: Ravi K
authors.initials: RK
authors.lastname: Ghanta
authors.affiliation: Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas; Department of Cardiovascular Surgery, Texas Heart Institute, Houston, Texas.
authors.forename: Susan Y
authors.initials: SY
authors.lastname: Green
authors.affiliation: Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas; Department of Cardiovascular Surgery, Texas Heart Institute, Houston, Texas.
authors.forename: Matt D
authors.initials: MD
authors.lastname: Price
authors.affiliation: Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas; Department of Cardiovascular Surgery, Texas Heart Institute, Houston, Texas.
authors.forename: Courtney C
authors.initials: CC
authors.lastname: Arredondo
authors.affiliation: Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas; Department of Cardiovascular Surgery, Texas Heart Institute, Houston, Texas.
authors.forename: D'Arcy
authors.initials: D
authors.lastname: Wainwright
authors.affiliation: Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas; Cardiovascular Research Institute, Baylor College of Medicine, Houston, Texas; Department of Cardiovascular Surgery, Texas Heart Institute, Houston, Texas; CHI St. Luke's Health-Baylor St. Luke's Medical Center, Houston, Texas.
authors.forename: Ourania
authors.initials: O
authors.lastname: Preventza
authors.affiliation: Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas; Cardiovascular Research Institute, Baylor College of Medicine, Houston, Texas; Department of Cardiovascular Surgery, Texas Heart Institute, Houston, Texas; CHI St. Luke's Health-Baylor St. Luke's Medical Center, Houston, Texas.
authors.forename: Kim I
authors.initials: KI
authors.lastname: de la Cruz
authors.affiliation: Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas; Department of Cardiovascular Surgery, Texas Heart Institute, Houston, Texas; CHI St. Luke's Health-Baylor St. Luke's Medical Center, Houston, Texas.
authors.forename: Muhammad
authors.initials: M
authors.lastname: Aftab
authors.affiliation: Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas; Cardiovascular Research Institute, Baylor College of Medicine, Houston, Texas; Department of Cardiovascular Surgery, Texas Heart Institute, Houston, Texas; CHI St. Luke's Health-Baylor St. Luke's Medical Center, Houston, Texas. Electronic address: slemaire@bcm.edu.
authors.forename: Scott A
authors.initials: SA
authors.lastname: LeMaire
authors.affiliation: Division of Cardiothoracic Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Texas; Cardiovascular Research Institute, Baylor College of Medicine, Houston, Texas; Department of Cardiovascular Surgery, Texas Heart Institute, Houston, Texas; CHI St. Luke's Health-Baylor St. Luke's Medical Center, Houston, Texas.
authors.forename: Joseph S
authors.initials: JS
authors.lastname: Coselli
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D017545:Aortic Aneurysm, Thoracic / Q000209:etiology / Q000401:mortality* / Q000601:surgery*; D019917:Blood Vessel Prosthesis Implantation; D005260:Female; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000150:complications* / Q000401:mortality / Q000601:surgery; D008875:Middle Aged; D011788:Quality of Life; D012189:Retrospective Studies; D016019:Survival Analysis; D015996:Survival Rate; D016896:Treatment Outcome; D055815:Young Adult