Neurodevelopment in children with hypoplastic left heart syndrome.

OBJECTIVE: To assess neurodevelopment in children with hypoplastic left heart syndrome (HLHS) or other types of functionally single ventricle (univentricular heart, UVH) and to estimate the effect of possible clinical predictors on outcome.

STUDY DESIGN: A total of 22 patients with HLHS and 14 with UVH, surviving after palliative surgery performed in the same center, and 42 healthy control subjects were examined at a median age of 30.2 months neurologically and according to the Bayley Scales of Infant Development in a population based prospective neurodevelopmental follow-up study.

RESULTS: The mean Mental Developmental Index was significantly lower (89.9) in patients with HLHS than in control subjects (105.5, P<.001), whereas there was no difference between patients with UVH (98.5) and control subjects. The mean Psychomotor Developmental Index in patients with HLHS (80.7, P<.001) as well as in those with UVH (94.5, P=.016) was significantly inferior to that in control subjects (105.3).

CONCLUSIONS: In patients with HLHS, mean Mental Developmental Index lags behind control subjects. Psychomotor Developmental Index is impaired in patients with both HLHS and UVH. Routine neurodevelopmental follow-up is recommended for this seriously ill patient group.