M. Catherin Freier

Relationship of Surgical Approach to Neurodevelopmental Outcomes in Hypoplastic Left Heart Syndrome

OBJECTIVE. Two strategies for surgical management are used for infants with hypoplastic left heart syndrome (HLHS), primary heart transplantation and the Norwood procedure. We sought to determine how these 2 surgical approaches influence neurodevelopmental outcomes at school age. METHODS. A multicenter, cross-sectional study of neurodevelopmental outcomes among school-aged children (>8 years of age) with HLHS was undertaken between July 2003 and September 2004. Four centers enrolled 48 subjects, of whom 47 completed neuropsychologic testing. Twenty-six subjects (55%) had undergone the Norwood procedure and 21 (45%) had undergone transplantation, with an intention-to-treat analysis. The mean age at testing was 12.4 ± 2.5 years. Evaluations included the Wechsler Abbreviated Scale of Intelligence, Clinical Evaluation of Language Fundamentals, Wechsler Individual Achievement Test, and Beery-Buktenica Developmental Test of Visual-Motor Integration. RESULTS. The mean neurocognitive test results were significantly below population normative values. The mean full-scale IQ for the entire cohort was 86 ± 14. In a multivariate model, there was no association of surgical strategy with any measure of developmental outcome. A longer hospital stay, however, was associated significantly with lower verbal, performance, and full-scale IQ scores. Aortic valve atresia was associated with lower math achievement test scores. CONCLUSIONS. Neurodevelopmental deficits are prevalent among school-aged children with HLHS, regardless of surgical approach. Complications that result in prolonged hospitalization at the time of the initial operation are associated with neurodevelopmental status at school age.

MR spectroscopy: Predicting long-term neuropsychological outcome following pediatric TBI

To identify useful acute indicators of long‐term neurocognitive outcome beyond clinical variables for children and adolescents treated for a traumatic brain injury (TBI).

Predicting neuropsychologic outcome after traumatic brain injury in children

The ability to predict long-term neurologic and neuropsychologic outcomes in 22 children, ages 1 week to 14 years at the time of traumatic brain injury, was investigated using proton magnetic resonance spectroscopy acquired post injury and compared with standardized neurologic, intellectual, and neuropsychologic testing done 1-7 years later. Clinical indicators of acute injury severity including age at injury, electroencephalography, spectroscopy metabolite ratio variables (N-acetyl aspartate/choline, choline/creatine) and lactate presence accurately classified children as functioning above or below the average range for most intellectual and neuropsychologic outcome measures. Combined clinical and spectroscopy variables accounted for approximately 50% of the variance in cognitive and neuropsychologic outcome confirming the validity of their predictive use. Of the injury severity indictors, presence of lactate is a particularly important prognostic marker of poor long-term intellectual and neuropsychologic functioning. Our findings indicate the potential for providing accurate estimates of long-term intellectual and neuropsychologic function after traumatic brain injury in infants and children using proton magnetic resonance spectroscopy in combination with clinical variables.

Developmental Outcomes After Pediatric Heart Transplantation

BACKGROUND Pediatric heart transplantation has now been successfully performed for more than 20 years. As survival rates have improved, more attention is now focused on long-term outcomes. METHODS This report reviews the literature on developmental outcomes after pediatric heart transplantation. RESULTS Pediatric patients undergoing heart transplantation generally can be expected to have developmental outcomes in the low-normal range, consistent with outcomes seen in other children with complex congenital heart disease requiring surgical intervention. When these children reach school age, or return to school, most can be expected to function reasonably well in mainstream school settings. A significant minority will require additional educational assistance. Approximately 10% will have significant neurologic impairment. In school, particular attention should be paid to evaluating the child for deficits in arithmetic and verbal skills. Performance may be better than predicted from IQ testing. Behavioral issues are common, with depression, concerns about social competence, and attention difficulties most frequently endorsed. This may pre-date transplantation in those who undergo transplantation during childhood and may improve with time. Parents more often report problem behaviors than teachers. Family resources and family coping skills are also strongly correlated with the childs emotions and coping skills. CONCLUSION The pediatric heart transplant recipients ability to transition from childhood into a happy and productive adult life can be significantly affected by his or her cognitive abilities, learning experiences, sense of self, and emotions. Attention to these factors is an important part of caring for these children.

Developmental outcomes and cognitive functioning in infant and child heart transplant recipients

Pediatric heart transplantation has become a mainstay in the treatment of end-stage heart disease in infants and children. There is, however, sparse information on post-transplant developmental and cognitive functioning. At Loma Linda University Childrens Hospital 223 infants have undergone heart transplantation surgery with a survival rate of 73% (n=165). This article reports on four areas of investigation of this cohort of infants. Infant development: Two studies were done utilizing the Bayley Scales of Infant Development (mean 100, S.D.+/-15). The first study (n=48) showed mean values within the normal range [Mental Development Index (MDI) 87; Psychomotor Developmental Index (PDI) 90]. The second study (n=23) showed developmental scores within normal limits in 4-8 month olds with a tendency for decline in development at 12-24 months (MDI 83, PDI 77). Child development: Ninty-one infant recipients were greater than 5 years old. Forty-five children were excluded because of long distances from the hospital, second transplantation, abnormal karyotype, primary language non-English, or invalid testing. The Wechsler Preschool and Primary Scale of Intelligence-Revised testing of 5-6-year-olds (n=23) showed a Full Scale IQ of 74, Performance IQ of 76, and Verbal IQ of 77. The Wechsler Intelligence Scale for Children-III testing of 7-10-year-olds (n=23) showed a Full Scale IQ of 86, Performance IQ of 89, and Verbal IQ of 86. The Wechsler Individual Achievement Test (mean 100, S.D.+/-15) yielded a Total Composite of 91, Mathematics Composite of 86, Language Composite of 98, and a Reading Composite of 94. Visual spatial skills: Visual motor integration (mean 100, S.D.+/-15) was evaluated in 5-10-year-olds with a mean of 87; however, 52% of the children had scores below 1 S.D. Subtests from the Wechsler scales that assess visual motor and visual spatial skills indicated significant deficits. Behavior: Younger children (n=33) demonstrated behaviors indicative of social isolation. Older children (n=36) showed behavior that was within the normal limits, but depression was noted in a significant number of them. Conclusions: Infant heart transplant recipients demonstrate IQ and achievement levels within the normal range, but there is a significant amount of variability with more children than would be expected scoring in the lower ranges. Children with heart transplantation are at risk for visual spatial skill deficits. Young children are at risk for social isolation while symptoms of depression are noted in older children.

The process of research in international settings: From risk assessment to program development and intervention

Although there are many challenges, international HIV prevention research and program evaluation are critical to advances in the health and welfare of people around the globe. While there is an increasing amount of literature describing outcomes of international prevention programs, there is sparse information regarding the process of developing and implementing international research. This brief report describes key elements in the development of collaborative international prevention research and programmatic implementation.