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Background: The purpose of this study was to assess deep hypothermic circulatory arrest (DHCA) as a modifier of neurodevelopmental (ND) outcomes in preschool children after cardiac surgery in infancy for repair of congenital heart defects (CHD)., Methods: This is a planned analysis of infants enrolled in a prospective study of apolipoprotein E polymorphisms and ND outcome after cardiac surgery. The effect of DHCA was assessed in patients with single or biventricular CHD without aortic arch obstruction. Neurodevelopmental assessment at 4 years of age included cognition, language, attention, impulsivity, executive function, social competence, and visual-motor and fine-motor skills. Patient and procedural variables were evaluated in univariate and multivariate models., Results: Neurodevelopmental testing was completed in 238 of 307 eligible patients (78%). Deep hypothermic circulatory arrest was used at the discretion of the surgeon at least once in 92 infants (38.6%) with a median cumulative duration of 36 minutes (range, 1 to 132 minutes). By univariate analysis, DHCA patients were more likely to have single-ventricle CHD (p = 0.013), lower socioeconomic status (p < 0.001), a higher incidence of preoperative ventilation (p < 0.001), and were younger and smaller at the first surgery (p < 0.001). By multivariate analysis, use of DHCA was not predictive of worse performance for any ND outcome., Conclusions: In this cohort of children undergoing repair of CHD in infancy, patients who underwent DHCA had risk factors associated with worse ND outcomes. Despite these, use of DHCA for repair of single-ventricle and biventricular CHD without aortic arch obstruction was not predictive of worse performance for any ND domain tested at 4 years of age., (Copyright © 2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: The prevalence of perioperative stroke in infants undergoing operations for congenital heart disease has not been well described. The objectives of this study were to determine the prevalence of stroke as assessed by postoperative brain magnetic resonance imaging (MRI), characterize the neuroanatomic features of focal ischemic injury, and identify risk factors for its development., Methods: Brain MRI was performed in 122 infants 3 to 14 days after cardiac operation with cardiopulmonary bypass, with or without deep hypothermic circulatory arrest. Preoperative, intraoperative, and postoperative data were collected. Risk factors were tested by logistic regression for univariate and multivariate associations with stroke., Results: Stroke was identified in 12 of 122 patients (10%). Strokes were preoperative in 6 patients and possibly intraoperative or postoperative in the other 6 patients, and were clinically silent except in 1 patient who had clinical seizures. Arterial-occlusive and watershed infarcts were identified with equal distribution in both hemispheres. Multivariate analysis identified lower birth weight, preoperative intubation, lower intraoperative hematocrit, and higher blood pressure at admission to the cardiac intensive care unit postoperatively as significant factors associated with stroke. Prematurity, younger age at operation, duration of cardiopulmonary bypass, and use of deep hypothermic circulatory arrest were not significantly associated with stroke., Conclusions: The prevalence of stroke in infants undergoing operations for congenital heart disease was 10%, half of which occurred preoperatively. Most were clinically silent and undetected without neuroimaging. Mechanisms included thromboembolism and hypoperfusion, with patient-specific, procedure-specific, and postoperative contributions to increased risk.

Background: Hyperglycemia has been associated with worse outcome after traumatic brain injury and cardiac surgery in adults. It is not known whether postoperative hyperglycemia results in worse neurodevelopmental outcome after infant cardiac surgery., Methods: Secondary analysis of postoperative glucose levels was performed in infants younger than 6 months of age enrolled in a prospective study of genetic polymorphisms and neurodevelopmental outcomes who were undergoing repair of two-ventricle cardiac defects. Neurodevelopmental outcomes at 1 year of age were assessed with the Bayley Scales of Infant Development-II, yielding two indices: Mental Developmental Index and Psychomotor Developmental Index., Results: Surgical repair was performed in 247 infants with 1 in-hospital and 3 late deaths. Neurodevelopmental evaluation was performed in 188 of 243 (77%) survivors. Glucose levels at cardiac intensive care unit admission and during the first 48 postoperative hours were available for 180 of 188 patients. Mean admission glucose was 328 +/- 106 mg/dL; maximum glucose was 340 +/- 109 mg/dL. At least one glucose was greater than 200 mg/dL in 160 of 180 patients, and 49 of 180 patients (27%) had a glucose greater than 400 mg/dL. Only 1 patient had a glucose less than 50 mg/dL. Female sex (p = 0.02), but no other patient or operative variable, was associated with higher glucose levels. Mean Mental Developmental Index and Psychomotor Developmental Index were 90.6 +/- 14.9 and 81.6 +/- 17.2, respectively. Hyperglycemia was not associated with lower Mental Developmental Index and Psychomotor Developmental Index scores for the entire cohort or for neonates alone., Conclusions: Hyperglycemia is common early after infant cardiac surgery, but is not associated with worse neurodevelopmental outcome at 1 year of age.

Objective: To perform a detailed, contemporary temporal-spatial characterization of neonatal seizures (NS) and to compare conventional EEG (CEEG) to single-channel EEG for NS detection., Methods: Digitally recorded CEEGs were reviewed for NS characteristics (quantity, duration, location of onset, peak-to-peak amplitude). The presence and characteristics of each NS were simultaneously noted in a single, derived EEG channel (C3-->C4)., Results: Eight hundred fifty-one seizures from 125 CEEGs recorded were analyzed. Mean seizure rate was 7.0 NS/h (range: 0.5-21). Mean seizure burden (percent time CEEG showed NS at any location) was 24.8% (range: 0.7-86.9). Seizure rate was only moderately correlated with seizure burden (Spearman coefficient=0.58). Eighty-one percent of NS originated from central-temporal or midline vertex electrodes. Seventy-eight percent of NS appeared in the C3-->C4 channel., Conclusions: Accurate measurement of NS burden requires detailed temporal-spatial characterization. The theoretical ceiling of sensitivity for NS detection in the single EEG channel C3-->C4 is high. However, further processing the raw EEG in limited electrode arrays may reduce the sensitivity of NS detection., Significance: CEEG is the gold standard for NS detection. However, reduced montage EEG techniques are increasingly available. This detailed contemporary temporal-spatial characterization of NS evaluates the potential limitations of reduced montage techniques.

We reviewed our computerized neonatal EEG database for records judged to display excessive positive temporal sharp waves (PTS) to determine their electroclinical associations and significance. Typical infants with excessive PTS were: (1) mature, with a mean conceptional age of 41.2 weeks, and (2) neurologically ill, judged by their high rate of associated EEG background abnormality (37 of 46; 80%) and clinical signs of encephalopathy (19 of 29; 66%). In comparison, healthy age-matched control infants never had excessive PTS. We consider an excess of PTS to be pathologic since they arise out of an abnormal EEG background in children exposed to potentially serious central nervous system illnesses and are associated with a high incidence of cerebral structural abnormalities (16 of 25; 64%). PTS may be comparable to the positive rolandic or vertex sharp waves in premature infants with periventricular injury of the deep white matter.

We report our observations of serial clinical and EEG examinations in 3 neonates during recovery from acute severe encephalopathy due to citrullinemia. Their electroclinical picture closely resembles the clinical stages of experimental models of hyperammonemia in monkeys. The length of the EEG interburst interval, a quantitative measure of EEG background abnormality, correlated with elevated serum levels of ammonia and suggests that hyperammonemia itself is a key figure in the genesis of encephalopathy in this condition. Finally, the manner in which the EEG normalizes during recovery from hyperammonemia in this setting suggests that burst-suppression resembles an exaggerated regression to the discontinuity of the very premature infant.

Seventy-eight electroencephalograms (EEGs) recorded from 44 premature infants with documented intraventricular hemorrhage (IVH) were retrospectively reviewed. Positive rolandic sharp waves (PRS) were observed in 13 of 44 (29.5%) patients. Among infants with grades 3 and 4 IVH, the incidence of PRS was 60% (9 of 15). During the period of study, typical PRS appeared in two EEGs of one premature infant who did not suffer an IVH. PRS were usually associated with moderately abnormal EEG backgrounds and with grades 3 and 4 IVH. Beta activity was superimposed on an average of 21.5% of PRS (beta-PRS). Infants with grades 3 and 4 IVH had a higher incidence of beta-PRS than those with smaller hemorrhages. PRS first appeared in early postnatal life, increased in abundance and then eventually disappeared from the records by 3-4 weeks of age. The field of distribution of PRS was centered at the central parasagittal region (Cz) with generous anteroposterior spread into the frontal and parietal vertices (Fz and Pz) and symmetric lateral spread into the rolandic areas (C3 and C4). Asymmetric involvement of PRS at C3 and C4 may signify differential hemispheric injury. The majority of infants whose EEGs contained PRS died or suffered serious neurologic sequelae. However, their outcomes were not significantly inferior to those without PRS.

Congenital Fiber Type Disproportion (CFTD) has recently been described as a consistent and stereotyped clinicopathological entity, including congenital nonprogressive hypotonia and weakness, contractures, kyphoscoliosis, high arched palate, dislocated hips, short stature, and feet deformities. Our personal experience with this condition suggests a wider disparity in the physical appearance and associated abnormalities of affected individuals than the well-defined clinical syndrome previously described. We are presenting 5 cases, including 2 siblings, whose muscle biopsies satisfy the major histological and statistical criteria for the diagnosis. Although each child clearly had hypotonia and weakness consistent with a congenital myopathy, only 3 had a sufficient number of other similarities to establish the diagnosis clinically. The clinical spectrum of the other cases ranged from one infant whose only abnormality was mild hypotonia in the legs to another whose problems included severe motor impairment, marked mental retardation, growth failure, frontal bossing, abnormal hair, and scoliosis. Even in retrospect, the diagnosis of CFTD could not have been supported on clinical grounds alone. Therefore, CFTD is a congenital myopathy whose diagnosis can be made only by muscle biopsy, rather than a distinct syndrome whose diagnosis can be assumed on the basis of clinical characteristics alone.