Early results of neurodevelopment following hybrid stage I for hypoplastic left heart syndrome.

Abstract

Motor skills and neurodevelopment in infants with hypoplastic left heart syndrome (HLHS) who have undergone Hybrid Stage I palliation is unknown. The purpose of this study is to assess early neurodevelopment in infants with HLHS after Hybrid Stage I palliation. Developmental assessment was performed in HLHS infants who underwent Hybrid Stage I palliation at 2 and 4months of age using the Test of Infant Motor Performance, and at 6months of age, prior to undergoing the second staged surgery, using the Bayley Scales of Infant and Toddler Development, 3rd edition (Bayley-III). Results were compared to healthy control subjects and norm-referenced data. The HLHS group scored between -1 and -2 standard deviations (SD) below the mean at 2months of age (p=0.002), and within -1 SD of the mean, at 4months of age (p=0.0019), on the TIMP. Compared to the control group, composite motor skills were significantly lower at 6months of age on the Bayley-III in the HLHS group (p=0.0489), however, not significant for cognitive (p=0.29) or language (p=0.68). Percentile rank motor scores were 17±20% in the HLHS group compared to 85±12% for the healthy age-matched control group. Infants with HLHS who undergo Hybrid Stage I palliation score lower on standardized motor skill tests compared to healthy age-matched controls and the norm-referenced population. This suggests that infants with HLHS have poorer motor skill performance than typically developing infants at 6months of age.