The type, frequency, and clinical presentation of developmental brain anomalies in 41 infants with the hypoplastic left heart syndrome are reviewed from the Children’s Hospital of Philadelophia, and the University of Pennsylvania School of Medicine, Philadelphia. A major or minor central nervous system abnormality occurred in 29% and included agenesis of the corpus callosum (3 cases), holoprosencephaly (1), micrencephaly (9), and cortical mantle malformation (8). Congenital brain anomalies were present in patients either with or without external dysmorphic features that involved the head, face and eyes, fingers, lung and kidney. In patients living only one day or less approximately 50% had recognizable malformations, microcephaly, and pulmonary anomalies. 
COMMENT. The authors recommend that infants with hypoplastic left heart syndrome deserve careful genetic, ophthalmologic and neurologic evaluations, including CT scan and long-term neurologic follow-up. In the same issue of Pediatrics the authors review their experience with acquired brain lesions associated with hypoplastic left heart syndrome, finding 45% with hypoxic, ischemic and hemorrhagic lesions secondary to CNS perfusion and glucose oxygen delivery. A duration of cardiopulmonary bypass with hypothermic total circulatory arrest longer than 40 minutes was associated with a higher incidence of acquired neuropathology. 
New management strategies for perinatal hypoxic-ischemic encephalopathy (HIE) are reviewed by Vannucci RC . Between 10% and 60% of asphyxiated neonates with HIE expire during the neonatal period. Of those who survive, 25% exhibit neuropsychologic deficits.