title: Twenty-year surgical experience with congenital supravalvar aortic stenosis.
abstract: BACKGROUND
  Congenital supravalvar aortic stenosis (SVAS) is an arteriopathy associated with Williams-Beuren syndrome and other elastin gene deletions. Our objectives were to review outcomes of congenital SVAS repair and to compare prosthetic patch repair techniques to all-autologous slide aortoplasty.
  
  
  METHODS
  Congenital SVAS repairs from 1988 to 2008 were retrospectively reviewed. Peak instantaneous gradients were estimated by Doppler interrogation. Variables were compared by either Student's t test or Fisher's exact test. Risk factors were analyzed by chi(2) test. Survival was estimated by the Kaplan-Meier method.
  
  
  RESULTS
  Of 25 primary SVAS repairs, there were 10 all-autologous slide aortoplasties and 15 prosthetic patch aortoplasties. The prosthetic patch group included the Doty technique (n = 9), patch-augmented slide aortoplasty (n = 3), modified Brom technique (n = 1), interposition graft (n = 1), and two-sinus patch with transverse arch augmentation (n = 1). There was 1 early and 1 late death. Cumulative survival for all patients was 96% at 5 and 10 years. Event-free survival did not differ between groups (p = 0.481). There were 2 late reoperations (both were prosthetic patch patients with bicuspid aortic valve: 1 with recurrent aortic valve stenosis and 1 with aortic insufficiency). Bicuspid aortic valve was the only risk factor for reoperation (p = 0.003). Three patients weighing less than 10 kg with diffuse disease underwent attempted slide aortoplasty: 2 required patch augmentation and 1 had a recurrent gradient in less than 1 year postoperatively.
  
  
  CONCLUSIONS
  Outcomes after SVAS repair were good by any technique. No advantage to all-autologous slide aortoplasty was apparent at current follow-up. Based on our experience, slide aortoplasty is not recommended for small patients with diffuse disease.
authors.affiliation: The Children's Hospital Heart Institute and University of Colorado Denver Health Sciences Center, Aurora, Colorado, USA.
authors.forename: Daniel J
authors.initials: DJ
authors.lastname: Scott
authors.forename: David N
authors.initials: DN
authors.lastname: Campbell
authors.forename: David R
authors.initials: DR
authors.lastname: Clarke
authors.forename: Steven P
authors.initials: SP
authors.lastname: Goldberg
authors.forename: Daniel R
authors.initials: DR
authors.lastname: Karlin
authors.forename: Max B
authors.initials: MB
authors.lastname: Mitchell
mesh_terms: D000293:Adolescent; D001013:Aorta, Thoracic / Q000601:surgery; D021921:Aortic Stenosis, Supravalvular / Q000000981:diagnostic imaging / Q000401:mortality / Q000601:surgery*; D001024:Aortic Valve Stenosis / Q000000981:diagnostic imaging / Q000401:mortality / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D004452:Echocardiography; D005260:Female; D006330:Heart Defects, Congenital / Q000000981:diagnostic imaging / Q000401:mortality / Q000601:surgery*; D006439:Hemodynamics / Q000502:physiology; D006801:Humans; D008297:Male; D019919:Prosthesis Implantation / Q000379:methods / Q000401:mortality; D012189:Retrospective Studies; D016019:Survival Analysis; D017741:Survivors; D016896:Treatment Outcome; D018980:Williams Syndrome / Q000000981:diagnostic imaging / Q000601:surgery; D055815:Young Adult