Developmental outcome of patients with hypoplastic left heart syndrome treated with heart transplantation.

Objective: To describe the outcome of children treated for hypoplastic left heart syndrome (HLHS) with heart transplantation.

Study Design: We evaluated outcomes in 26 children treated for HLHS in a single center; 13 children were evaluated with the Bayley Scales of Infant Development, Child Behavior Checklist (CBCL), and Vineland Adaptive Behavior Scales (VABS), and 13 were seen after 36 months of age and were evaluated with the Wechsler Preschool and Primary Scale of Intelligence, CBCL, and VABS at 36 to 72 months or the Wechsler Intelligence Scale for Children-III, CBCL, and VABS for those older than 72 months of age.

Results: Bayley Scales of Infant Development results revealed a median Mental Developmental Index of 88 (range <50 to 102) and a Psychomotor Developmental Index of 86.5 (<50 to 113), both significantly lower than expected in the general population. Intelligence quotient results on either the Wechsler Preschool and Primary Scale of Intelligence or Wechsler Intelligence Scale for Children-III were also significantly lower than expected, with a mean verbal score of 90.5 +/- 12.4, performance score of 88.9 +/- 14.5, and full scale score of 88.5 +/- 13.0. On the Vineland scales, 39% scored >1 SD below the mean on measures of daily living scales, 22% on the socialization subscale, 48% on the communication subscale, and 52% on the adaptive behavior scale.

Conclusions: In this small population of children treated for HLHS with heart transplantation, both cognitive deficits and adaptive/behavioral abnormalities are described. Early identification with appropriate referral for services could potentially enhance the outcomes for these children.