title: Surgical repair of supravalvular aortic stenosis in children with williams syndrome: a 30-year experience.
abstract: BACKGROUND
  Williams syndrome is an uncommon genetic disorder associated with supravalvular aortic stenosis (SVAS) in childhood. We reviewed outcomes of children with Williams syndrome who underwent repair of SVAS during a 30-year period at a single institution.
  
  
  METHODS
  Between 1982 and 2012, 28 patients with Williams syndrome were operated on for SVAS. Mean age at operation was 5.2 years (range, 3 months to 13 years), and mean weight at operation was 18.6 kg (range, 4.1 to 72.4 kg). Associated cardiac lesions in 11 patients (39.3%) were repaired at the time of the SVAS repair. The most common associated cardiac lesion was main pulmonary artery stenosis (8 of 28 [28%]).
  
  
  RESULTS
  A 3-patch repair was performed in 10 patients, a Doty repair in 17, and a McGoon repair in 1 (3.6%). There were no early deaths. Follow-up was 96% complete (27 of 28). Overall mean follow-up was 11.2 years (range, 1 month to 27.3 years). Mean follow-up was 5 years (range, 1 month to 14.3 years) for the 3-patch repair patients and 14.7 years (range, 6 weeks to 27 years) for the Doty repair patients. Of the 17 Doty patients, there were 4 (24%) late deaths, occurring at 6 weeks, 3.5 years, 4 years, and 16 years after the initial operation. There were no late deaths in the 3-patch repair patients. Overall survival was 86% at 5, 10, and 15 years after repair. Survival was 82% at 5, 10 and 15 years for the Doty repair patients. Overall, 6 of 27 patients (22%) patients required late reoperation at a mean of 11.2 years (range, 3.6 to 23 years). No 3-patch repair patients required reoperation. Overall freedom from reoperation was 91% at 5 years and 73% at 10 and 15 years. Freedom from reoperation for the Doty repair patients was 93% at 5 years and 71% at 10 and 15 years.
  
  
  CONCLUSIONS
  Surgical repair of SVAS in children Williams syndrome has excellent early results. However, significant late mortality and morbidity warrants close follow-up.
authors: Departments of Cardiac Surgery and Cardiology, Royal Children's Hospital, Melbourne, Victoria, Australia; University of Melbourne, Melbourne, Victoria, Australia.
authors: Tyson A
authors: TA
authors: Fricke
authors: Departments of Cardiac Surgery and Cardiology, Royal Children's Hospital, Melbourne, Victoria, Australia; University of Melbourne, Melbourne, Victoria, Australia; Murdoch Children's Research Institute, Melbourne, Victoria, Australia.
authors: Yves
authors: Y
authors: d'Udekem
authors: Departments of Cardiac Surgery and Cardiology, Royal Children's Hospital, Melbourne, Victoria, Australia; University of Melbourne, Melbourne, Victoria, Australia; Murdoch Children's Research Institute, Melbourne, Victoria, Australia.
authors: Christian P
authors: CP
authors: Brizard
authors: Women's and Children's Hospital, Adelaide, South Australia, Australia.
authors: Gavin
authors: G
authors: Wheaton
authors: Departments of Cardiac Surgery and Cardiology, Royal Children's Hospital, Melbourne, Victoria, Australia; University of Melbourne, Melbourne, Victoria, Australia; Murdoch Children's Research Institute, Melbourne, Victoria, Australia.
authors: Robert G
authors: RG
authors: Weintraub
authors: Departments of Cardiac Surgery and Cardiology, Royal Children's Hospital, Melbourne, Victoria, Australia; University of Melbourne, Melbourne, Victoria, Australia; Murdoch Children's Research Institute, Melbourne, Victoria, Australia. Electronic address: igor.konstantinov@rch.org.au.
authors: Igor E
authors: IE
authors: Konstantinov
mesh_terms: D000293:Adolescent; D021921:Aortic Stenosis, Supravalvular / Q000000981:diagnostic imaging / Q000401:mortality / Q000601:surgery*; D058385:Cardiac Valve Annuloplasty / Q000009:adverse effects / Q000379:methods* / Q000401:mortality; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D016208:Databases, Factual; D015150:Echocardiography, Doppler / Q000379:methods; D004502:Education, Medical, Continuing; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D008297:Male; D011183:Postoperative Complications / Q000503:physiopathology / Q000601:surgery; D012086:Reoperation; D012189:Retrospective Studies; D018570:Risk Assessment; D012720:Severity of Illness Index; D013021:South Australia; D015996:Survival Rate; D016896:Treatment Outcome; D018980:Williams Syndrome / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*