Background: Congenital heart disease (CHD) is the most common of all birth defects and is the leading cause of infant morbidity and mortality (American Heart Association, 2010). Hypoplastic left heart syndrome (HLHS) is 100% fatal without palliation. Despite increased survival, since a surgical strategy was developed nearly 30 years ago, neurodevelopmental outcomes and quality of life are poor. The purpose of this study is to examine cerebral blood flow and neurodevelopment of infants born with HLHS after Hybrid Stage I palliation.
Methods: HLHS infants who underwent Hybrid Stage I palliation and healthy age-matched control subjects underwent transcranial Doppler at baseline, 2, 4, and 6 months of age. Systolic, diastolic, and mean velocities, as well as pulsatility index in the middle cerebral artery was recorded. Developmental assessment was performed at 2 and 4 months, using the Test of Infant Motor Performance (TIMP), and at 6 months of age, or prior to undergoing the second staged surgical repair, using the Bayley Scales of Infant and Toddler Development, 3rd edition and results were compared.
Results: The HLHS group scored lower compared to controls on the TIMP at 2 months of age (p=0.002), -1 to -2 standard deviation (SD) below the norm, and 4 months of age (p=0.0019), within -1 SD of the norm. Motor skills were significantly lower in the HLHS group compared with controls (p=0.049), however not significant for cognitive (p=0.29) or language (p=0.68) at 6 months. There was no significant correlation between transcranial Doppler velocities and cognitive, language, or motor skills at 6 months of age.
Conclusions: Infants with HLHS who undergo Hybrid Stage I palliation score lower on standardized testing when compared to normal controls and the norm-referenced population. Cerebral blood flow velocity did not predict neurodevelopmental outcomes.