title: Association of impaired linear growth and worse neurodevelopmental outcome in infants with single ventricle physiology: a report from the pediatric heart network infant single ventricle trial.
abstract: OBJECTIVE
  To describe neurodevelopmental outcomes in infants with single ventricle (SV) physiology and determine factors associated with worse outcomes.
  
  
  METHODS
  Neurodevelopmental outcomes for infants with SV enrolled in a multicenter drug trial were assessed at 14 months of age using the Bayley Scales of Infant Development-II. Multivariable regression analysis was used to identify factors associated with worse outcomes.
  
  
  RESULTS
  Neurodevelopmental testing was performed at 14 ± 1 months in 170/185 subjects in the trial. Hypoplastic left heart syndrome was present in 59% and 75% had undergone the Norwood operation. Mean Psychomotor Developmental Index (PDI) and mental developmental index (MDI) were 80 ± 18 and 96 ± 14, respectively, (normal 100 ± 15, P < .001 for each). Group-based trajectory analysis provided a 2-group model ("high" and "low") for height z-score trajectory and brain type natriuretic peptide (BNP) trajectory. The predicted PDI scores were 15 points higher in the "high" height z-score trajectory compared with the "low" cluster (P < .001). A higher number of serious adverse events during the trial was associated with lower PDI scores (P = .02). The predicted MDI scores were 13-17 points lower in "low height trajectory-high BNP trajectory" group compared with the other 3 groups (P < .001). MDI scores were also lower in subjects who required extracorporeal membrane oxygenation during the neonatal hospitalization (P = .01) or supplemental oxygen at discharge (P = .01).
  
  
  CONCLUSIONS
  Neurodevelopmental outcome at 14 months of age is impaired in infants with SV physiology. Low height trajectory and high BNP trajectory were associated with worse neurodevelopmental outcomes. Efforts to improve nutritional status alone may not improve neurodevelopmental outcomes.
authors.affiliation: Department of Pediatrics, Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA. Ravishankar@email.chop.edu
authors.forename: Chitra
authors.initials: C
authors.lastname: Ravishankar
authors.forename: Victor
authors.initials: V
authors.lastname: Zak
authors.forename: Ismee A
authors.initials: IA
authors.lastname: Williams
authors.forename: David C
authors.initials: DC
authors.lastname: Bellinger
authors.forename: J William
authors.initials: JW
authors.lastname: Gaynor
authors.forename: Nancy S
authors.initials: NS
authors.lastname: Ghanayem
authors.forename: Catherine D
authors.initials: CD
authors.lastname: Krawczeski
authors.forename: Daniel J
authors.initials: DJ
authors.lastname: Licht
authors.forename: Lynn
authors.initials: L
authors.lastname: Mahony
authors.forename: Jane W
authors.initials: JW
authors.lastname: Newburger
authors.forename: Victoria L
authors.initials: VL
authors.lastname: Pemberton
authors.forename: Richard V
authors.initials: RV
authors.lastname: Williams
authors.forename: Renee
authors.initials: R
authors.lastname: Sananes
authors.forename: Amanda L
authors.initials: AL
authors.lastname: Cook
authors.forename: Teresa
authors.initials: T
authors.lastname: Atz
authors.forename: Svetlana
authors.initials: S
authors.lastname: Khaikin
authors.forename: Daphne T
authors.initials: DT
authors.lastname: Hsu
mesh_terms: D002657:Child Development; D002658:Developmental Disabilities / Q000188:drug therapy / Q000453:epidemiology / Q000209:etiology* / Q000503:physiopathology*; D004311:Double-Blind Method; D005260:Female; D006128:Growth; D006352:Heart Ventricles / Q000002:abnormalities*; D006801:Humans; D007223:Infant; D008297:Male; D011446:Prospective Studies; D016896:Treatment Outcome