title: Valve-sparing aortic root replacement in Loeys-Dietz syndrome.
abstract: BACKGROUND
  Loeys-Dietz syndrome (LDS) is a recently recognized aggressive aortic disorder characterized by root aneurysm, arterial tortuosity, hypertelorism, and bifid uvula or cleft palate. The results of prophylactic root replacement using valve-sparing procedures (valve-sparing root replacement [VSRR]) in patients with LDS is not known.
  
  
  METHODS
  We reviewed all patients with clinical and genetic (transforming growth factor-β receptor mutations) evidence of LDS who underwent VSRR at our institution. Echocardiographic and clinical data were obtained from hospital and follow-up clinic records.
  
  
  RESULTS
  From 2002 to 2009, 31 patients with a firm diagnosis of LDS underwent VSRR for aortic root aneurysm. Mean age was 15 years, and 24 (77%) were children. One (3%) patient had a bicuspid aortic valve. Preoperative sinus diameter was 3.9±0.8 cm (z score 7.0±2.9) and 2 (6%) had greater than 2+ aortic insufficiency. Thirty patients (97%) underwent reimplantation procedures using a Valsalva graft. There were no operative deaths. Mean follow-up was 3.6 years (range, 0 to 7 years). One patient required late repair of a pseudoaneurysm at the distal aortic anastomosis, and 1 had a conversion to a David reimplantation procedure after a Florida sleeve operation. No patient suffered thromboembolism or endocarditis, and 1 (3%) patient experienced greater than 2+ late aortic insufficiency. No patient required late aortic valve repair or replacement.
  
  
  CONCLUSIONS
  Loeys-Dietz syndrome is an aggressive aortic aneurysm syndrome that can be addressed by prophylactic aortic root replacement with low operative risk. Valve-sparing procedures have encouraging early and midterm results, similar to those in Marfan syndrome, and are an attractive option for young patients.
authors.affiliation: Johns Hopkins Division of Cardiac Surgery, Department of Surgery, The Johns Hopkins Hospital, Baltimore, Maryland 21287, USA.
authors.forename: Nishant D
authors.initials: ND
authors.lastname: Patel
authors.forename: George J
authors.initials: GJ
authors.lastname: Arnaoutakis
authors.forename: Timothy J
authors.initials: TJ
authors.lastname: George
authors.forename: Jeremiah G
authors.initials: JG
authors.lastname: Allen
authors.forename: Diane E
authors.initials: DE
authors.lastname: Alejo
authors.forename: Harry C
authors.initials: HC
authors.lastname: Dietz
authors.forename: Duke E
authors.initials: DE
authors.lastname: Cameron
authors.forename: Luca A
authors.initials: LA
authors.lastname: Vricella
mesh_terms: D000293:Adolescent; D000328:Adult; D017541:Aneurysm, False / Q000000981:diagnostic imaging / Q000601:surgery; D001011:Aorta / Q000000981:diagnostic imaging / Q000601:surgery*; D001807:Blood Vessel Prosthesis; D019917:Blood Vessel Prosthesis Implantation / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D004452:Echocardiography; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D055947:Loeys-Dietz Syndrome / Q000000981:diagnostic imaging / Q000235:genetics / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D009154:Mutation; D011093:Polyethylene Terephthalates; D011183:Postoperative Complications / Q000000981:diagnostic imaging / Q000601:surgery; D011474:Prosthesis Design; D017755:Prosthesis Fitting; D018125:Receptors, Transforming Growth Factor beta / Q000235:genetics; D012086:Reoperation; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: D011093:Polyethylene Terephthalates; D018125:Receptors, Transforming Growth Factor beta