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Objective: Evaluate the impact of near-term delivery on neurodevelopmental (ND) outcomes in children with congenital heart disease (CHD)., Methods: Secondary analysis of data from a study of genetic polymorphisms and ND outcomes after cardiac surgery in infants. The effect of gestational age (GA) as a continuous variable on ND outcomes was evaluated using general linear regression models. GA was also evaluated as a categorical variable to seek a threshold for better outcomes. ND domains tested at 4 years of age included cognition, language skills, attention, impulsivity, memory, executive function, social competence, visual-motor, and fine-motor skills., Results: ND outcomes and GA were available for 378 infants. Median GA was 39 weeks (range, 28-42 weeks) with 351 born at 36 weeks or more (near-term/term). In univariate analysis of the near-term/term subgroup, older GA predicted better performance for cognition, visual-motor, and fine-motor skills. After covariate adjustment, older GA predicted better performance for fine-motor skills (P = .018). Performance for cognition, language, executive function, social skills, visual-motor, and fine-motor skills was better for those born at 39 to 40 weeks of GA or more versus those born at less than 39 weeks (all P < .05)., Conclusions: These findings are consistent with the hypothesis that delivery before 39 to 40 weeks of GA is associated with worse outcomes in patients with CHD. Early delivery of a child with CHD is often indicated because of maternal or fetal health issues. In the absence of these concerns, these data suggest that elective (or spontaneous) delivery at 39 to 40 weeks of GA is associated with better ND outcomes., (Copyright © 2012 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Objective We sought to define the neurodevelopmental status of school-aged survivors of total anomalous pulmonary venous connection repaired during infancy. Methods All school-aged survivors of total anomalous pulmonary venous connection repair performed at a single institution were eligible. Thirty children returned for neurologic examination and neurodevelopmental testing. Results The median age at total anomalous pulmonary venous connection repair was 16 days (range, 1-141 days), and age at testing was 11 years (range, 6-19 years). Pulmonary venous return was supracardiac in 14 patients, infracardiac in 12 patients, cardiac in 3 patients, and mixed in 1 patient. Preoperative obstructed total anomalous pulmonary venous connection was present in 6 patients. Circulatory arrest was used in all repairs, with a median duration of 35 minutes (range, 17-55 minutes). At follow-up, microcephaly (head circumference <5%) was present in 28%. Neuromuscular examination was suspect or abnormal in 27%. Mean Full-scale IQ (95.3 +/- 18.5) and Verbal IQ (98.6 +/- 20.2) were not different from population norms, but Performance IQ (92.3 +/- 16.9) was significantly lower than population norms ( P = .02). Fine motor skills and visual-motor coordination were significantly impaired ( P < .01 for Grooved Pegboard and Test of Visual-Motor Integration). Patients with total anomalous pulmonary venous connection also had difficulty with tests of attention (Test of Everyday Attention for Children, P < .01), but results of tests of memory function were not significantly different from population norms. Conclusions School-aged survivors of infant total anomalous pulmonary venous connection repair exhibit a significant incidence of neurodevelopmental difficulties. Fine motor function, visual-motor integration, and attention are the most commonly affected domains. Evaluation of these children is indicated to identify those who are at risk for learning disabilities and who could benefit from early intervention.

Objective: To evaluate cerebral cortical function with electroencephalography in infants of cocaine-addicted mothers., Design: Patient series., Setting: The Children's Hospital of Philadelphia (Pa)., Participants: Thirty-five consecutive infants of cocaine-addicted mothers hospitalized for a comprehensive health assessment and 51 healthy, age-matched infants studied with electroencephalography and respiratory thermistor because they were siblings of sudden infant death victims (comparison group)., Interventions: None., Measurements/main Results: Behavioral states during spontaneous daytime sleep were classified as active sleep or quiet sleep; quiet sleep was further characterized as immature, tracé alternant sleep or mature, continuous, slow wave sleep. No episodes of ictal apnea or nonictal apnea were recorded in infants of cocaine-addicted mothers; nonictal apnea was observed in one control patient. No electrographic seizures were recorded. There were no significant differences between the proportions of infants exposed to cocaine in utero and that of controls who displayed excessive sharp electroencephalographic transients, background abnormalities, immaturity, and hypermaturity. Electroclinical sleep discordance was present in 5.7% of infants of cocaine-addicted mothers vs 0% of controls. Cocaine-exposed infants displayed mature, continuous, slow wave sleep below 45 weeks of conceptional age in a significantly higher percentage than those in the comparison group., Conclusions: Although frank electroencephalographic abnormalities were infrequent in infants whose mothers were addicted to cocaine, they differed significantly in their younger age of onset of continuous, slow wave sleep. Our findings provide continued reason for concern that infants of cocaine-addicted mothers may suffer subtle adverse neurologic, cognitive, or behavioral effects later in life. The longitudinal assessment of sleep disturbance and its relation to later development might permit tracking of the long-term effects of prenatal exposure to cocaine.

This study details the type, frequency, and clinical presentation of developmental brain anomalies in 41 infants with the hypoplastic left heart syndrome encountered during a 52-month interval. Overall, 29% of the infants had either a major or minor central nervous system abnormality. Overt central nervous system malformations, including 3 cases of agenesis of the corpus callosum and 1 case of holoprosencephaly, were seen in 4 infants (10%). Micrencephaly (brain weight at autopsy more than 2 SDs below the mean for age) was found in 27% of the infants. An immature cortical mantle was seen in 21% of the study group. Seven infants (17%) had specific recognizable patterns of malformation. The absence of dysmorphic physical features did not preclude overt or subtle central nervous system malformations. Conversely, the presence of dysmorphic features did not reliably indicate an underlying brain anomaly. Infants who had hypoplastic left heart syndrome as one of multiple nonneurologic malformations were more likely to have micrencephaly than those infants with hypoplastic left heart syndrome as an isolated abnormality. Occurrence of developmental neuropathology was elevated in those infants with hypoplastic left heart syndrome who did not have a recognizable pattern of malformation but who were small for gestational age, microcephalic, or had ocular abnormalities. Infants with hypoplastic left heart syndrome deserve careful genetic, opthalmologic, and neurologic evaluations, imaging of their intracranial anatomy, and long-term neurologic follow-up.