title: Relationship of surgical approach to neurodevelopmental outcomes in hypoplastic left heart syndrome.
abstract: OBJECTIVE
  Two strategies for surgical management are used for infants with hypoplastic left heart syndrome (HLHS), primary heart transplantation and the Norwood procedure. We sought to determine how these 2 surgical approaches influence neurodevelopmental outcomes at school age.
  
  
  METHODS
  A multicenter, cross-sectional study of neurodevelopmental outcomes among school-aged children (>8 years of age) with HLHS was undertaken between July 2003 and September 2004. Four centers enrolled 48 subjects, of whom 47 completed neuropsychologic testing. Twenty-six subjects (55%) had undergone the Norwood procedure and 21 (45%) had undergone transplantation, with an intention-to-treat analysis. The mean age at testing was 12.4 +/- 2.5 years. Evaluations included the Wechsler Abbreviated Scale of Intelligence, Clinical Evaluation of Language Fundamentals, Wechsler Individual Achievement Test, and Beery-Buktenica Developmental Test of Visual-Motor Integration.
  
  
  RESULTS
  The mean neurocognitive test results were significantly below population normative values. The mean full-scale IQ for the entire cohort was 86 +/- 14. In a multivariate model, there was no association of surgical strategy with any measure of developmental outcome. A longer hospital stay, however, was associated significantly with lower verbal, performance, and full-scale IQ scores. Aortic valve atresia was associated with lower math achievement test scores.
  
  
  CONCLUSIONS
  Neurodevelopmental deficits are prevalent among school-aged children with HLHS, regardless of surgical approach. Complications that result in prolonged hospitalization at the time of the initial operation are associated with neurodevelopmental status at school age.
authors.affiliation: Children's Healthcare of Atlanta, Atlanta, GA 30322-1062, USA. mahlew@kidsheart.com
authors.forename: William T
authors.initials: WT
authors.lastname: Mahle
authors.forename: Karen J
authors.initials: KJ
authors.lastname: Visconti
authors.forename: M Catherin
authors.initials: MC
authors.lastname: Freier
authors.forename: Stephen M
authors.initials: SM
authors.lastname: Kanne
authors.forename: William G
authors.initials: WG
authors.lastname: Hamilton
authors.forename: Angela M
authors.initials: AM
authors.lastname: Sharkey
authors.forename: Richard E
authors.initials: RE
authors.lastname: Chinnock
authors.forename: Kathy J
authors.initials: KJ
authors.lastname: Jenkins
authors.forename: Peter K
authors.initials: PK
authors.lastname: Isquith
authors.forename: Thomas G
authors.initials: TG
authors.lastname: Burns
authors.forename: Pamela C
authors.initials: PC
authors.lastname: Jenkins
mesh_terms: D000293:Adolescent; D006348:Cardiac Surgical Procedures / Q000009:adverse effects; D002648:Child; D002658:Developmental Disabilities / Q000175:diagnosis* / Q000209:etiology; D005260:Female; D016027:Heart Transplantation / Q000009:adverse effects; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000601:surgery*; D008297:Male; D009483:Neuropsychological Tests; D010166:Palliative Care; D011594:Psychometrics