title: Midterm outcome of valve-sparing aortic root replacement in inherited connective tissue disorders.
abstract: BACKGROUND
  This study determined the midterm outcome of valve-sparing aortic root replacement for patients with inherited connective tissue disorders.
  
  
  METHODS
  From 1993 to 2008, 94 patients underwent valve-sparing aortic root replacement. Sixty patients (64%), average age 33 years (range, 15 to 61 years), had inherited connective tissue disorders: Marfan syndrome, 54 (92%); Loeys-Dietz syndrome, 5 (8%); and smooth muscle α-actin (ACTA2) mutation in 1. Median preoperative sinus diameter was 52 mm (range, 42 to 76 mm), and moderate/severe aortic regurgitation was present in 14 (23%). Seven (12%, 1993 to 1999) underwent remodeling procedures, and 53 had reimplantation procedures. Cusp repair was performed in 4. Median follow-up was 55 months (range, 1 to 149 months). There were 15 patients in the early term (1993 to 2000) and 45 in the late term (2001 to 2008).
  
  
  RESULTS
  Four late deaths occurred (cardiac, 3; aortic, 1), with 10-year survival of 86%. Rates of freedom from aortic valve replacement at 5 and 10 years were 85% and 58% in remodeling and 96% and 58% in reimplantation. Risk factors for reoperations were postprocedure intraoperative aortic insufficiency greater than mild (p = 0.046), remodeling procedure (p = 0.016), and early term (p = 0.0002). One patient (2%) with none/trivial postprocedure aortic insufficiency required aortic valve replacement. Freedom from reoperation in patients with none/trivial postprocedure aortic insufficiency at 5 and 10 years was 100% and 67%.
  
  
  CONCLUSIONS
  Meticulous control of aortic insufficiency during operation would bring favorable midterm durability in valve-sparing aortic root replacement using a reimplantation technique, even in patients with inherited connective tissue disorders.
authors: Department of Cardiovascular Surgery, National Cardiovascular Center, Osaka, Japan. hirtanak@hsp.ncvc.go.jp
authors: Hiroshi
authors: H
authors: Tanaka
authors: Hitoshi
authors: H
authors: Ogino
authors: Hitoshi
authors: H
authors: Matsuda
authors: Kenji
authors: K
authors: Minatoya
authors: Hiroaki
authors: H
authors: Sasaki
authors: Yutaka
authors: Y
authors: Iba
mesh_terms: D000199:Actins / Q000235:genetics; D000293:Adolescent; D000328:Adult; D001021:Aortic Valve / Q000601:surgery*; D006349:Heart Valve Diseases / Q000209:etiology / Q000601:surgery*; D006801:Humans; D055947:Loeys-Dietz Syndrome / Q000150:complications; D008382:Marfan Syndrome / Q000150:complications; D008875:Middle Aged; D009154:Mutation; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: C541116:ACTA2 protein, human; D000199:Actins