Prenatal head growth and white matter injury in hypoplastic left heart syndrome

Children with hypoplastic left heart syndrome (HLHS) have fit increased prevalence of central nervous system (CNS) abnormalities. The extent to which this problem is due to CNS maldevelopment. prenatal ischemia, postnatal chronic cyanosis and/or multiple exposures to cardiopulmonary bypass is unknown. To better Understand the etiolooy of CNS abnormalities in HLHS, we evaluated 68 neonates with HI-FIS: in 28 cases. both fetal Ultrasound and echocardiogram data were available to assess head size, head growth and aortic valve anatomy (atresia of, stenosis). In addition, we evaluated neuropathology in 11 electively aborted HLHS fetuses. The mean head circumference percentile in HLHS neonates was significantly smaller than HLHS fetuses (22 +/- 21% versus 40 +/- 4%. p < 0.001). A significant decrease in head growth. defined as a 50% reduction in head circumference percentile, was observed in half (14/28) of HLHS fetuses and nearly a quarter (6/28) were already growth restricted (<= 10%) at the time of initial evaluation. Brains from HLHS fetuses demonstrated chronic diffuse white matter injury of varying severity. These patterns of prenatal head growth and brain histopathology identify a spectrum of abnormal CNS development and/or injury in HLHS fetuses.