Factors associated with neurodevelopment for children with single ventricle lesions

J Pediatr. 2014 Sep;165(3):490-496.e8. doi: 10.1016/j.jpeds.2014.05.019. Epub 2014 Jun 19.


Objective: To measure neurodevelopment at 3 years of age in children with single right-ventricle anomalies and to assess its relationship to Norwood shunt type, neurodevelopment at 14 months of age, and patient and medical factors.

Study design: All subjects in the Single Ventricle Reconstruction Trial who were alive without cardiac transplant were eligible for inclusion. The Ages and Stages Questionnaire (ASQ, n = 203) and other measures of behavior and quality of life were completed at age 3 years. Medical history, including measures of growth, feeding, and complications, was assessed through annual review of the records and phone interviews. The Bayley Scales of Infant Development, Second Edition (BSID-II) scores from age 14 months were also evaluated as predictors.

Results: Scores on each ASQ domain were significantly lower than normal (P < .001). ASQ domain scores at 3 years of age varied nonlinearly with 14-month BSID-II. More complications, abnormal growth, and evidence of feeding, vision, or hearing problems were independently associated with lower ASQ scores, although models explained <30% of variation. Type of shunt was not associated with any ASQ domain score or with behavior or quality-of-life measures.

Conclusion: Children with single right-ventricle anomalies have impaired neurodevelopment at 3 years of age. Lower ASQ scores are associated with medical morbidity, and lower BSID-II scores but not with shunt type. Because only a modest percentage of variation in 3-year neurodevelopmental outcome could be predicted from early measures, however, all children with single right-ventricle anomalies should be followed longitudinally to improve recognition of delays.

Trial registration: ClinicalTrials.gov NCT00115934.

Publication types

  • Randomized Controlled Trial
  • Research Support, N.I.H., Extramural

MeSH terms

  • Child, Preschool
  • Developmental Disabilities / etiology*
  • Female
  • Heart Defects, Congenital / complications*
  • Heart Defects, Congenital / surgery*
  • Heart Ventricles / abnormalities*
  • Heart Ventricles / surgery*
  • Humans
  • Infant
  • Male
  • Nervous System / growth & development*
  • Prospective Studies

Associated data

  • ClinicalTrials.gov/NCT00115934