title: Outcomes after operations for bicuspid aortic valve disease in the pediatric population.
abstract: BACKGROUND
  Outcomes after operations for bicuspid aortic valve disease in pediatric patients were determined.
  
  
  METHODS
  Between 1977 and 2011, 146 consecutive patients underwent surgical repair of bicuspid aortic valve. Median age at operation was 207 days (range, 5 days to 16 years). Indication for surgery was stenosis in 113, insufficiency in 25, and both in 8. Valve debridement was done in 76 patients, and complex repairs in 70, including 61 who required addition of pericardial patches, consisting of the creation of a neocommissure in 55, cusp extension in 33, and a perforation repair in 6. The valve was made tricuspid in 38 patients (29 cusp extensions).
  
  
  RESULTS
  Twenty-year survival was 88% (95% confidence interval, 73% to 95%). After a mean follow-up of 8 ± 7 years, 35 patients needed a reintervention. Freedom from reintervention at 18 years was 43% (95% confidence interval, 28% to 56%). At the latest follow-up, an additional 13 patients without reoperation had moderate or severe stenosis, and 17 had moderate regurgitation. Seventy-eight patients had an event-free long-term outcome (no reintervention, stenosis, or regurgitation). The only independent predictive factors of an event-free outcome were not having addition of patch material at repair (hazard ratio, 12; p = 0.05) and shorter bypass time (HR, 1.01; p = 0.023). The 10-year freedom from any significant event was 60% (95% confidence interval, 46% to 71%) for those without use of patch material, whereas nearly all those with a patch repair had an adverse event at that time.
  
  
  CONCLUSIONS
  Outcomes after surgical repair of bicuspid aortic valves in the pediatric population are excellent, especially if the repair can be performed without the addition of patches. Primary repair should be offered because long-lasting results can be achieved if the disease can be relieved by simple procedures.
authors.affiliation: Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Victoria, Australia.
authors.forename: Javariah
authors.initials: J
authors.lastname: Siddiqui
authors.forename: Christian P
authors.initials: CP
authors.lastname: Brizard
authors.forename: Igor E
authors.initials: IE
authors.lastname: Konstantinov
authors.forename: John
authors.initials: J
authors.lastname: Galati
authors.forename: Gavin
authors.initials: G
authors.lastname: Wheaton
authors.forename: Michael
authors.initials: M
authors.lastname: Cheung
authors.forename: Stephen
authors.initials: S
authors.lastname: Horton
authors.forename: Yves
authors.initials: Y
authors.lastname: d'Udekem
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006349:Heart Valve Diseases / Q000401:mortality / Q000601:surgery*; D019918:Heart Valve Prosthesis Implantation; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008297:Male; D008943:Mitral Valve / Q000601:surgery*; D012189:Retrospective Studies; D012307:Risk Factors; D015996:Survival Rate / Q000639:trends; D016896:Treatment Outcome; D014739:Victoria / Q000453:epidemiology
keywords: 20