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PubMed2006

Maternal-fetal surgery for myelomeningocele: neurodevelopmental outcomes at 2 years of age.

OBJECTIVE: This study was undertaken to examine short-term neurodevelopmental outcomes in children with myelomeningocele (MMC) who underwent in utero neurosurgical closure. STUDY DESIGN: Between 1998 and 2002, 51 fetuses underwent in utero MMC closure at our Center. Thirty (63%) of these children have returned for neurodevelopmental testing at 2 years of age using the Bayley Scales of Infant Development and Preschool Language Scales. RESULTS: Overall shunt rate was 43% in this group. Neurodevelopmental testing found 67% with cognitive language and personal-social skills in the normal range, 20% with mild delays, and 13% with significant delays. Children with shunted hydrocephalus scored lower than those with unshunted ventriculomegaly. CONCLUSION: Children who have undergone fetal MMC closure have characteristic neurodevelopmental deficits that do not appear worsened by fetal surgery, and developmental outcomes may be improved by decreasing the need for ventriculoperitoneal shunting.

Child Development↗
PubMed2005

Early brain injury in premature newborns detected with magnetic resonance imaging is associated with adverse early neurodevelopmental outcome.

OBJECTIVE: To determine the neurodevelopmental outcome of prematurely born newborns with magnetic resonance imaging (MRI) abnormalities. STUDY DESIGN: A total of 89 prematurely born newborns (median age 28 weeks postgestation) were studied with MRI when stable for transport to MRI (median age, 32 weeks postgestation); 50 newborns were studied again near term age (median age, 37 weeks). Neurodevelopmental outcome was determined at 18 months adjusted age (median) using the Mental Development Index (Bayley Scales Infant Development II) and a standardized neurologic exam. RESULTS: Of 86 neonatal survivors, outcome was normal in 51 (59%), borderline in 22 (26%), and abnormal in 13 (15%). Moderate/severe MRI abnormalities were common on the first (37%) and second (32%) scans. Abnormal outcome was associated with increasing severity of white matter injury, ventriculomegaly, and intraventricular hemorrhage on MRI, as well as moderate/severe abnormalities on the first (relative risk [RR] = 5.6; P = .002) and second MRI studies (RR = 5.3; P = .03). Neuromotor abnormalities on neurologic examination near term age (RR = 6.5; P = .04) and postnatal infection (RR = 4.0; P = .01) also increased the risk for abnormal neurodevelopmental outcome. CONCLUSIONS: In premature newborns, brain abnormalities are common on MRI early in life and are associated with adverse neurodevelopmental outcome.

Brain Injuries↗
PubMed2005

Neurodevelopmental outcomes in congenital hypothyroidism: comparison of initial T4 dose and time to reach target T4 and TSH.

OBJECTIVES: To compare neurodevelopmental outcomes in severe and moderate congenital hypothyroidism (CH) among 3 different initial L-thyroxine doses and to examine the effect of the time to thyroid function normalization on neurodevelopmental outcomes. STUDY DESIGN: Neurodevelopmental assessments of 31 subjects included the Mullen Scales of Early Learning, Wechsler Preschool and Primary Scale of Intelligence-Revised, Wechsler Intelligence Scale for Children, Wide-Range Achievement Test, and Child Behavioral Checklist. RESULTS: Subjects started on higher initial L-thyroxine doses (50 mug) had full-scale IQ scores 11 points higher than those started on lower (37.5 mug) initial doses. However, verbal IQ, performance IQ, and achievement scores did not differ among the 3 treatment cohorts. Subjects with moderate CH had higher full-scale IQ scores than subjects with severe CH, regardless of the initial treatment dose. Subjects who took longer than 2 weeks to normalize thyroid function had significantly lower cognitive, attention, and achievement scores than those who achieved normal thyroid function at 1 or 2 weeks of therapy. CONCLUSIONS: Initial L-thyroxine dose and faster time to normalization of thyroid function are important to optimal neurodevelopmental outcome. In severe CH, it is important to choose an initial dose at the higher end of the recommended range to achieve these goals.

Analysis of Variance↗
PubMed2006

Neurodevelopmental outcome after early repair of a ventricular septal defect with or without aortic arch obstruction.

OBJECTIVES: Cross-sectional studies of intermediate-term survivors of infant cardiac surgery have revealed a high frequency of neurodevelopmental disabilities. Few data exist regarding neurodevelopmental outcome of infants undergoing surgical intervention for a ventricular septal defect. The purpose of this study was to evaluate the neurodevelopmental outcome at 1 year of age of children who had surgical repair in infancy of a ventricular septal defect or a ventricular septal defect with aortic arch obstruction. METHODS: Children who underwent repair of a ventricular septal defect or single-stage repair of a ventricular septal defect with aortic arch obstruction at less than 6 months of age were assessed at 1 year of age by using the Bayley Scales of Infant Development II, which yields the Mental Development Index and the Psychomotor Development Index, both with an expected mean of 100 +/- 15. RESULTS: At 1 year, 55 patients (ventricular septal defect alone = 36; ventricular septal defect with aortic arch obstruction = 19) returned for evaluation. The mean Mental Development Index was 92.6 +/- 11.7, with 3 (5%) patients scoring 70 or less. The mean Psychomotor Development Index was 86.1 +/- 16.4, with 10 (18%) patients scoring 70 or less. Patients with a suspected or confirmed genetic syndrome had both a lower Mental Development Index score (P = .011) and a lower Psychomotor Development Index score (P = .001). Mental Development Index and Psychomotor Development Index were independent of anatomic (specifically aortic arch obstruction) and intraoperative factors (specifically deep hypothermic circulatory arrest). CONCLUSIONS: Neurodevelopmental outcome at 1 year of age was within the normal limits for most patients who underwent repair of a ventricular septal defect or a ventricular septal defect with aortic arch obstruction during infancy.

Aortic Arch Syndromes↗
PubMed2006

Development and validation of a test battery to assess subtle neurodevelopmental differences in children.

There is increasing concern over the impact of low-dose exposures to environmental chemicals on children's neurobehavioral function. To determine subtle alterations in children's function, it is necessary to move beyond global measures such as IQ and employ tests that can detect small, subtle neurodevelopmental effects across a broad array of behavioral domains. We investigated the sensitivity and specificity of a battery of 63 neurodevelopmental tests or tasks designed to detect outcomes representing the type of subtle neurodevelopmental deficits caused by exposure to neurotoxicants in school-aged children. We studied Neonatal Intensive Care Unit (NICU) graduates, a population known to be at risk for both major and mild anomalies in perception, motor functioning, learning, memory and cognition. This population served as a surrogate to evaluate the capacity of these tests and tasks to predict such deficits. The subjects' histories of previous exposures to any environmental neurotoxicants was not ascertainable, but exposures to elevated levels was not suspected. Over one-third of the 63 measures proved capable of detecting pre-diagnosed lower IQ, the presence of a learning disability (LD) or a neonatal risk profile with at least 70% sensitivity and specificity. Some tests were differentially sensitive and specific, depending upon the presence or absence of one or more of several covariates such as gender, age, hearing status, or familiarity with computers. Tests were also eliminated from the battery if they were affected by too many covariates. We propose calling the final battery of tests that are specific and sensitive to subtle neurodevelopmental changes the Rochester test battery (RTB). Further studies are needed to confirm the capability of the RTB to detect subtle changes associated with neurotoxic exposures.

Adolescent↗
PubMedDate not supplied

[Neurodevelopmental hypothesis in schizophrenia].

The hypothesis for a neurodevelopmental basis to the underlying physiopathological disorder leading to schizophrenia has been proposed by many investigators for more than two decades. This hypothesis is supported by -several lines of evidence. Pregnancy and delivery complications, particularly those with known or presumed impact on fetal neurologic development, result in increased risk for psychotic disorders. Other possible etiologic candidates include viral infections. Minor physical anomalies, manifesting as slight anatomical defects of the head, hair, eyes, mouth, hands and feet, as dematoglyphic fluctuating asymmetries, are due to some injury occurring during the first or second trimester of fetal life, and are more common among patients with schizophrenia and in their unaffected siblings than in the general population. But a major Issue in a such neurodevelopmental model theory is the delayed onset of the schizophrenic disorder. Although early signs and prodromal symptoms can be defined retrospectively in patients who have developed schizophrenia, they do have to be confirmed as early predictors in prospective and longitudinal studies. Abnormalities in brain development and maturation seem to begin prenatally, but may continue throughout childhood and the observed changes during these periods must have -consequences for the neuronal circuitry and connectivity. Advances in brain imaging have now led to the identification of a great number of brain abnormalities in schizophrenia. The most consistently replicated structural anomaly present in the brains of patients with chronic schizophrenia is ventricular enlargement. These findings also include medial temporal lobe structures (which include the amygdala, hippocampus, and parahippocampal gyrus), and neocortical temporal lobe regions (superior temporal gyrus). There is also some evidence for frontal lobe abnormalities, particularly prefrontal gray matter and orbitofrontal regions. Similarly, there are findings for parietal lobe abnormalities (particularly of the inferior parietal lobule which includes both supramarginal and angular gyri) and subcortical abnormalities (basal ganglia, corpus callosum, and thalamus) but more equivocal evidence for cerebellar abnormalities. However, it is possible that the brain structural abnormalities observed in schizophrenia are not only due to neurodevelopmental anomalies, but also to an alteration in cortical plasticity and maturation processes that occurs over the long course of the disease. The genetic predisposition for schizophrenia has been confirmed in many studies. It is utterly disappointing that molecular genetic approaches have so far not yielded conclusive evidence for vulnerability or protection genes in schizophrenia. Future studies will likely benefit from: 1) studying more homogeneous patient groups, 2) studying high risk populations such as biological relatives of patients with schizophrenia, 3) using longitudinal and prospective methodological design in order to confirm the predictive validity of neurodevelopmental clues found in patients with schizophrenia, 4) applying newer strategies such as composite phenotypes of developmental origin, in combination with new genetic methods.

Amygdala↗
PubMed1994

Severe respiratory failure in neonates: mortality and morbidity rates and neurodevelopmental outcomes.

OBJECTIVE: To compare the survival, neurodevelopmental, and health outcomes of children with severe respiratory illness treated with and without extracorporeal membrane oxygenation (ECMO). DESIGN: Prospective collection of clinical and demographic data of all neonates reaching illness severity criteria, with follow-up at 8 and 20 months of age. Patients were assigned to treatment by the attending physician. PATIENTS: Consecutive sample of 74 neonates during a 24-month period with an alveolar-to-arterial gradient exceeding 620 for 8 or more hours. RESULTS: Eighteen (69%) of 26 neonates treated with conventional therapy survived to 20 months, in comparison with 43 (90%) of 48 neonates treated with ECMO. The conventionally treated group had significantly more chronic lung disease, longer duration of oxygen therapy, more chronic reactive airway disease, and more rehospitalizations than those treated with ECMO. Hospital charges were similar in the two groups. Macrocephaly was noted in 24% of those treated with ECMO and in none of the conventional group. Of those completing evaluation, 4 (24%) of 17 conventionally treated survivors and 20 (26%) of 38 ECMO-treated survivors had neurodevelopmental impairment. CONCLUSION: Survivors of severe neonatal respiratory illness have significant pulmonary and neurodevelopmental impairment, regardless of the treatment used. Neonates treated with ECMO had neurodevelopmental outcomes similar to those of patients treated conventionally, but better pulmonary outcomes.

Chronic Disease↗
PubMed1996

Neurodevelopmental outcome at age two years amongst very low birth weight infants: results from a district general hospital.

STUDY OBJECTIVES: To describe the survival and neurodevelopmental outcome at age 2 years of very low birth weight infants from routinely collected information in a district general hospital setting. DESIGN: Case note review and child health surveillance information was collected on all liveborn < 1,501 g infants born in our district general hospital over 4 years, 1989-1992. Main outcome measures were (a) death before discharge, (b) normal neurodevelopment at age 2 years, (c) minor impairment at age 2 years, (d) major impairment at age 2 years. Comparability with other published work was examined. SETTING: Rotherham District General Hospital, a maternity unit with neonatal intensive care facilities. Selected infants were transferred for continuing neonatal intensive care to a tertiary unit. Most infants were followed up to age 2 years in a dedicated clinic. SUBJECTS: Liveborn very low birth weight infants. MAIN RESULTS: Over the 4 year period there were 125 liveborn infants < 1,501 g; 93 (74%) survived to discharge home. Defined neurodevelopmental outcome measures were easily retrievable in 92 children at age 2 years from hospital case notes or child health surveillance records. Sixty-four children (70%) were neurodevelopmentally normal, 10 children (11%) had a major impairment (mostly cerebral palsy) and 18 (19%) had an isolated minor impairment. CONCLUSIONS: The incidence of major impairment amongst very low birth weight infants in our district general unit is broadly comparable with other published series. Outcome measures of neurodevelopmental status are available from routinely collected clinical information. The use of such measures to make detailed comparisons between units is problematical because of a wide range of confounding variables.

Child, Preschool↗
PubMed1985

Effect of early neurodevelopmental therapy in normal and at-risk survivors of neonatal intensive care.

At a corrected age of 3 months, 80 low birth-weight infants were assigned to normal or at-risk groups on the basis of a neurodevelopmental assessment scale. Both groups were further divided into intervention and non-intervention sub-groups (20 infants in each). Intervention consisted of monthly hospital-based neurodevelopmental therapy in addition to a home exercise programme. Infants were re-assessed by a physiotherapist at 6, 9, and 12 months, and were tested at 12 months by an independent psychologist blinded for infant group. Mean birthweight and gestational age were similar for normal and at-risk groups. At-risk infants had higher mean neurodevelopmental scores throughout the study period and lower 1-year development quotients (DQ) than normals. In neither normal nor at-risk groups did neurodevelopmental therapy alter the pattern of development or the outcome.

Child Development↗
PubMed1982

A study of neurodevelopmental findings in early adolescent delinquents.

Subtle deficits or delays in the development of the central nervous system in young children are associated with problems affecting learning, behavior, and social adjustment. Less is known about the impact of such neurodevelopmental dysfunction in adolescents. This study assesses the possible association between neurodevelopmental delays and juvenile delinquency. An adolescent neurodevelopment examination was devised and administered to 54 delinquents and 51 secondary school students. Six areas were assessed. There were no significant differences in the prevalence of minor neurologic signs (P = 0.37) or in gross motor function (P = 0.02) and temporal sequential organization (P = 0.04). The greatest differences were in visual processing (P = 0.0002) and auditory-language function (P = 0.0001). Eighteen percent of delinquents and 4% of the comparison group were deficient in two or more neurodevelopmental areas. Nine percent of the delinquents and none of the comparison group had three or more dysfunctions. A neurodevelopmental examination may be a useful diagnostic tool for identifying endogenous factors in behavior, learning, and adjustment problems of this age group. Such findings have implications for the formulation of individualized management strategies.

Adolescent↗
PubMed2002

Neurodevelopmental outcome in children with fetal hydrocephalus.

Thirty-eight children with fetal hydrocephalus treated surgically during the neonatal period were analyzed concerning their neurodevelopmental outcome by means of a follow-up study. The mean gestation at diagnosis was 31.1 weeks, and the mean postconceptual age at the first operation was 36.3 weeks. The ages of the patients at the last follow-up ranged from 3.8 to 11.8 years (mean = 7.4 years of age). During the follow-up period, they underwent neurologic examinations and were also evaluated to determine the developmental quotient or intelligence quotient. The relationships between age at diagnosis or the first operation for hydrocephalus and neurodevelopmental outcome, and between the lateral ventricular width/hemispheral width ratio at diagnosis and age at diagnosis or the first operation, and neurodevelopmental outcome were statistically evaluated. The neurodevelopmental outcome in the patients was normal in three patients, borderline intelligence in one patient, mental retardation in seven patients, and motor disturbance in 27 patients (five of whom were intellectually normal). The etiology was a major determinant of the outcome in the children with early postnatal surgical treatment of fetal hydrocephalus. Furthermore, an early onset and a high lateral ventricular width/hemispheral width ratio at diagnosis of hydrocephalus were significantly correlated with a poor intellectual outcome.

Cerebral Palsy↗
PubMed1999

Premature sexual development in individuals with neurodevelopmental disabilities.

Studies on precocious puberty have primarily focused on children with typical patterns of growth and cognitive development. This study reviewed diagnostic data from the records of 15,719 patients with neurodevelopmental disabilities for diagnoses associated with premature sexual development/precocious puberty. Thirty-two individuals with premature sexual development were identified, with the earliest changes seen in one girl at 1 year 7 months of age. In this group, the mean age at onset was 7 years 2 months in boys and 5 years 11 months in girls. Central precocious puberty, which was the most common cause of onset of early pubertal changes, was present in 15 of the 32 children. The results of this study suggest that children with a neurodevelopmental disability are at increased risk of premature pubertal changes when compared to children without a neurodevelopmental disability. This study indicates the need for health-care providers to be vigilant in screening for early pubertal changes in children with neurodevelopmental disabilities.

Child↗
PubMed2004

Neurodevelopmental outcome at 8 months and 4 years among infants born full-term small-for-gestational-age.

OBJECTIVES: To examine the association between intrauterine growth restriction and neurodevelopmental outcome among full-term small-for-gestational-age (SGAT) infants at 8 months and 4 years of age. STUDY DESIGN: Growth parameters at birth and test scores on measures of neurodevelopmental function for 3922 children born SGAT were compared with those of 29,369 children born appropriately grown-for-gestational-age term from similar economic backgrounds. Additional within-SGAT/economic group comparisons were made for 1684 SGAT infants with symmetric undergrowth at birth and 2034 SGAT infants with asymmetric undergrowth at birth. RESULTS: Regardless of socioeconomic background, infants born SGAT were found to be at significantly increased risk for neurodevelopmental difficulties at 8 months and at 4 years of age. Few within SGAT/socioeconomic group differences in neurodevelopmental outcome appeared to be associated with specific pattern of growth restriction at birth. CONCLUSIONS: The present findings provide further evidence of the individual and public health impact of SGAT birth.

Adolescent↗
PubMed2004

Apnea is associated with neurodevelopmental impairment in very low birth weight infants.

OBJECTIVE: To determine whether apnea in preterm infants is associated with abnormal neurodevelopmental outcome. METHODS: We determined the number of days that apnea and bradycardia spells were noted by the nursing staff, during the initial hospitalization of 175 preterm infants of less than <1250 g birth weight or <32 weeks gestation who had been enrolled in the follow-up program of the Royal Victoria Hospital. Multiple logistic and multiple linear regression models were constructed to determine the relationships between apnea days and neurodevelopmental impairment at 3 years of age, after correcting for gestation, sex, intrauterine growth restriction, intraventricular hemorrhage, periventricular leukomalacia, pre- and postnatal steroids, and maternal education. RESULTS: A total of 41 infants had neurodevelopmental impairment (Bayley MDI or PDI <70, cerebral palsy or blindness). By multiple logistic regression, an increasing number of days on which at least one apnea occurred, "total apnea days", and male sex were significantly associated with increasing probability of neurodevelopmental impairment, p<0.01, the sum of days of assisted ventilation and apnea days occurring after extubation was also associated with impairment in a separate regression model, p<0.001. Lower MDI at 3 years was significantly associated with postnatal steroid use, p=0.004. Lower PDI was associated with increasing apnea days, male sex, and postnatal steroid use, p<0.001. Functional impairment (a score on any one of the four dimensions of the Vineland scale <70), found in 17% of the infants, was associated with increasing apnea days, p<0.05. Caffeine treatment was not independently associated with any outcome. CONCLUSION: An increasing number of days that apnea was recorded during hospitalization was associated with a worse outcome. Among the potential explanations for this finding is the possibility that multiple recurrent hypoxic and bradycardic spells may cause brain injury.

Apnea↗
PubMed2005

The neurodevelopmental model of schizophrenia: update 2005.

Neurodevelopmental models of schizophrenia that identify longitudinal precursors of illness have been of great heuristic importance focusing most etiologic research over the past two decades. These models have varied considerably with respect to specificity and timing of hypothesized genetic and environmental 'hits', but have largely focused on insults to prenatal brain development. With heritability around 80%, nongenetic factors impairing development must also be part of the model, and any model must also account for the wide range of age of onset. In recent years, longitudinal brain imaging studies of both early and adult (to distinguish from late ie elderly) onset populations indicate that progressive brain changes are more dynamic than previously thought, with gray matter volume loss particularly striking in adolescence and appearing to be an exaggeration of the normal developmental pattern. This supports an extended time period of abnormal neurodevelopment in schizophrenia in addition to earlier 'lesions'. Many subtle cognitive, motor, and behavioral deviations are seen years before illness onset, and these are more prominent in early onset cases. Moreover, schizophrenia susceptibility genes and chromosomal abnormalities, particularly as examined for early onset populations (ie GAD1, 22q11DS), are associated with premorbid neurodevelopmental abnormalities. Several candidate genes for schizophrenia (eg dysbindin) are associated with lower cognitive abilities in both schizophrenic and other pediatric populations more generally. Postmortem human brain and developmental animal studies document multiple and diverse effects of developmental genes (including schizophrenia susceptibility genes), at sequential stages of brain development. These may underlie the broad array of premorbid cognitive and behavioral abnormalities seen in schizophrenia, and neurodevelopmental disorders more generally. Increased specificity for the most relevant environmental risk factors such as exposure to prenatal infection, and their interaction with susceptibility genes and/or action through phase-specific altered gene expression now both strengthen and modify the neurodevelopmental theory of schizophrenia.

Adult↗
PubMed2003

Effect of breastmilk consumption on neurodevelopmental outcomes at 6 and 12 months of age in VLBW infants.

PURPOSE: To determine the influence of breastmilk consumption, as a dose response, in very low-birth-weight (VLBW) infants (< 1,500 g) on neurodevelopmental outcomes at 6 and 12 months corrected age, and to determine the influence of selected sociodemographic and infant variables on neurodevelopmental outcomes. SUBJECTS: VLBW infants (n = 148) who were fed mother's milk or formula by parental choice. DESIGN: Prospective cohort with longitudinal follow-up at 6 and 12 months corrected age. METHODS: Self-administered questionnaires given to mothers at study entry, before discharge, and at 3-, 6-, and 12-month follow-up visits. During hospitalization, mothers recorded the 24-hour volume of expressed milk once per week. At each follow-up visit, the volume of a single feeding was assessed by pre- and postbreastfeeding test weights of infants measured on an electronic scale accurate to 1.0 g. The amount of breastfeeding was also assessed by feeding records and mother's report. MAIN OUTCOME MEASURES: The Bayley Scales of Infant Development (2nd Edition), the Mental Developmental Index (MDI), and the Psychomotor Developmental Index (PDI). PRINCIPAL RESULTS: After controlling for specific sociodemographic and infant variables, this study of VLBW infants showed no statistically significant effect of predominantly breastfeeding compared with predominantly formula feeding on neurodevelopmental outcomes to 12 months corrected age. The most significant predictor of MDI scores at 6 and 12 months corrected age was birth weight, in which higher birth weights predicted higher MDI scores. CONCLUSIONS: Despite the lack of statistically significant differences, the findings suggest a small but consistent advantage in developmental scores in infants who were fed their mother's milk compared with those who were predominantly formula fed. Supporting parents to breastfeed preterm infants may maximize the potential advantages of early nutrition in the neurodevelopmental outcome of VLBW infants.

Breast Feeding↗
PubMed2000

Neurodevelopmental outcome of patients after the fontan operation: A comparison between children with hypoplastic left heart syndrome and other functional single ventricle lesions.

OBJECTIVE: To compare neurodevelopmental outcome (NDO) in patients with hypoplastic left heart syndrome (HLHS), other functional single ventricle lesions, and the standard population and to investigate predictors of NDO in the population of children with functional single ventricle (FSV). STUDY DESIGN: A time- and age-defined cohort of patients with the Fontan circulation was recruited to participate in neurodevelopmental testing, behavioral evaluation, and imaging of the central nervous system. The Wechsler Intelligence test was the primary measure of NDO. Analysis included comparison of patients with HLHS with other patients with functional single ventricles. Other potential clinical predictors of NDO were investigated. RESULTS: The mean Full Scale Wechsler Intelligence score was 101.4+/-5.4. For the HLHS subgroup the mean Full Scale Wechsler score was 93.8+/-7.3, and for the non-HLHS subgroup it was 107.0+/-7.0. Although the HLHS group had significantly lower scores than the non-HLHS subgroup, neither subgroup scored significantly different from the standard population on the Wechsler Scales. Socioeconomic status, circulatory arrest, and perioperative seizures also were predictive of neurodevelopmental outcome. CONCLUSION: Neurodevelopmental and behavioral outcome in patients who have undergone the Fontan procedure including patients with HLHS is good in the preschool and early school years, with Wechsler Intelligence scores generally in the normal range.

Central Nervous System↗
PubMed2002

Long-term neurodevelopmental outcomes in school-aged children after neonatal arterial switch operation.

OBJECTIVE: Neurodevelopmental status of children between 8 and 14 years of age after neonatal arterial switch operation for transposition of the great arteries has not previously been systematically evaluated. METHODS: Within a longitudinal study, 60 unselected children operated on as neonates with combined deep hypothermic circulatory arrest and low-flow cardiopulmonary bypass were reevaluated at the age of 7.9 to 14.3 years (mean +/- SD 10.5 +/- 1.6 years). Clinical neurologic status and standardized tests to assess gross motor function, intelligence, acquired abilities, language, and speech were carried out, and the results were related to preoperative, perioperative, and postoperative status, to management, and to neurodevelopmental status at a mean age of 5.4 years. RESULTS: Neurologic and speech impairments were evidently more frequent (27% and 40%, respectively) than in the general population. Intelligence and socioeconomic status were not different (P =.29 and P =.11), whereas motor function, acquired abilities, and language were reduced (P < or =.04 for each). Overall rate of developmental impairment in one or more domains was 55%, compared with 26% at age 5.4 years. Multivariable analysis showed that severe preoperative acidosis and hypoxia predicted reduced motor function (mean deficit 52.7 points, P <.001), whereas longer bypass duration predicted both neurologic (odds ratio per 10 minutes of bypass duration 1.8, P =.04) and speech (odds ratio per 10 minutes of bypass duration 1.9, P =.02) dysfunction, and perioperative and postoperative cardiocirculatory insufficiency predicted neurologic (odds ratio 6.5, P =.04) and motor (mean deficit 6.8 points, P =.03) dysfunction. CONCLUSIONS: The neonatal arterial switch operation with combined circulatory arrest and low-flow bypass is associated increasingly with age, with reduced neurodevelopmental outcome but not with cognitive dysfunction. In our experience, the risk of long-term neurodevelopmental impairment after neonatal corrective cardiac surgery is related to deleterious effects of the global perioperative management and to special adverse effects of prolonged bypass duration. Severe preoperative acidosis and hypoxia and postoperative hemodynamic instability must be considered as important additional risk factors.

Adolescent↗