Catherine Limperopoulos

Developmental and Functional Outcomes at School Entry in Children with Congenital Heart Defects

OBJECTIVEnTo describe developmental and functional outcomes of children with congenital heart defects (CHDs) at school entry after open heart surgery.nnnSTUDY DESIGNnInfants with CHDs who underwent surgical repair in infancy were recruited and assessed prospectively for developmental progress. At 5 years of age (64.2 +/- 11.3 months), 94 subjects were evaluated in a blind fashion by using a variety of standardized measures.nnnRESULTSnMean IQ scores were in the low average range (90-94). Receptive language was in the average range (103.6 +/- 14.4). Behavioral difficulties were common (27.1%), with internalizing problems being more frequent. Functional limitations in socialization (93.0 +/- 17.1), daily living skills (94.6 +/- 16.4), communication (90.0 +/- 14.1), and adaptive behavior (92.1 +/- 15.8) were noted in 11% to 17% of children. With the Functional Independence Measure for Children, 20% to 22% of subjects were more dependent than their peers in self-care and social cognition, although few (4.5%) had mobility restrictions. Predictors of developmental and functional limitations included: abnormal postoperative neurologic examination, microcephaly, deep hypothermic circulatory arrest time, palliation, acyanotic heart lesion, age at surgery, and maternal education.nnnCONCLUSIONSnAfter infant open-heart surgery, children with CHDs may exhibit a range of developmental difficulties at school entry that enhances risk for learning challenges and decreased social participation.

Health and well-being of children with congenital cardiac malformations, and their families, following open-heart surgery

Infants who survive open-heart surgery are at risk for developmental disability, which may impact on the well-being not only of the child, but also the family. The objective of our prospective study, therefore, was to determine the long-term health-related quality of life of children with congenital cardiac malformations following open-heart surgery, and to describe the persisting level of stress in their families. To this end, 49 parents completed the Child Health Questionnaire, the Parenting Stress Index, and the Child Behaviour Checklist as part of a developmental follow-up protocol when their child was 5 years of age. Mean scores on the Child Health Questionnaire were in the normal range, with physical well-being equal to 53.5, psychosocial well-being 50.9, with only 6.4 percent and 8.5 percent of subjects, respectively, falling within the suboptimal range of less than 40. The distribution of scores on the Parenting Stress Index, however, were more variable, with over one-quarter of parents indicating a high level of stress, with almost one-fifth having low levels of stress, and just over half scoring in the normal range, with the group mean being 52.6 plus or minus 32.3. An abnormal neurologic examination before surgery was associated with lower physical health (beta equal to -5.5, p equal to 0.02, r2 equal to 0.18), whereas lower arterial saturations of oxygen, less than 85 percent preoperatively, was associated with lower psychosocial health (beta equal to -6.6, p equal to 0.01, and r(2) equal to 0.14). The internalizing and externalizing behaviours of the child were significantly correlated with psychosocial well being, with r ranging from -0.32 to -0.52, and p less than 0.05. Parental stress also correlated with psychosocial health (r equal to -0.48 and p equal to 0.0009). Overall, the perception by the parents of the health-related quality of life of their child is favourable 5 years following open-heart surgery during infancy. Many parents, nonetheless, continue to feel either stressed or defensive about their child, particularly if their child exhibits behavioural difficulties. Our findings suggest that strategies need to be considered to enhance family well-being in the planning and delivery of health services to this population at high risk.

Developmental progress of children with congenital heart defects requiring open heart surgery

Recent advances for infants requiring early open heart surgery have resulted in a dramatic decline in mortality and severe morbidity. The developmental progress of these new survivors is currently being defined. Causes contributing to brain injury are multifactorial, and may involve preoperative, intraoperative, and postoperative events. Before surgery, these children often exhibit hypotonia, poor state regulation, microcephaly, and developmental delays. These findings are particularly prevalent in newborns. In the acute postoperative period, neurodevelopmental deficits continue to manifest clinically. Long-term follow-up studies indicate that subtle neurological deficits and global developmental lags are characteristic of this population. Overall, severe neurological sequelae are uncommon; however, mild to moderate developmental disabilities are prevalent. Functional limitations, academic achievements, and health-related quality of life are areas that deserve further attention.

Association Between Electroencephalographic Findings and Neurologic Status in Infants With Congenital Heart Defects

Neurologic status is of concern in infants with congenital heart defects undergoing open heart surgery. The association between perioperative electroencephalography (EEG) with acute neurologic status and subsequent outcome was examined in a cohort of 60 infants. Preoperative EEG and neurologic examinations were performed within 1 to 2 days prior to surgery (n = 27) and postoperatively (n = 47). Prior to surgery, 15 of 27 infants had normal EEG, whereas 5 had epileptiform activity and 9 had disturbances in background activity that were primarily moderate (8/9) and diffuse (7/9). Postoperatively, only 17 of 47 infants had normal recordings. Newborns (<1 month) were more likely (P < .001) to demonstrate EEG abnormalities than infants. Epileptiform activity was documented in 15, whereas 28 had background abnormalities that were moderate-severe (22/28) and diffuse (20/28) in most. Epileptiform activity prior to surgery was always associated with an abnormal neurologic examination, and this association persisted postoperatively (86%). Moderate to severe background abnormalities in the postoperative EEG was also strongly associated with acute neurologic abnormalities (93%). Severe background abnormalities (n = 5) were 100% predictive of death or severe disability. Long-term follow-up revealed that all children with normal postoperative EEGs had positive neurologic outcomes (P = .04); however, there were many false positives. Perioperative EEG abnormalities increased the likelihood for acute neurologic findings, whereas normal recordings following surgery were reassuring with regard to a favorable outcome. (J Child Neurol 2001;16:471-476).

Patterns of use of educational and rehabilitation services at school age for children with congenitally malformed hearts.

BACKGROUNDnInfants with congenitally malformed hearts who require early open-heart surgery are at high risk for developmental, psychosocial, and academic difficulties. Our objective was to describe the pattern of use of educational supports and rehabilitation services in these children at early school age.nnnMETHODSnParents of children who participated in a prospective study of developmental progress following open-surgery were contacted to participate in a telephone survey. The questionnaire included questions regarding current educational and rehabilitation resources their child was receiving, as well as the needs perceived by the parents for services, and obstacles to accessing services.nnnRESULTSnThe survey was completed by 60 families, the mean age of the children being 8.1 years, with standard deviation of 1.1 years. Of the children, 22% received educational supports, which primarily included supplemental tutoring. Rehabilitation services were received by 23%, speech therapy for 9 children, psychologic support for 6, occupational therapy for 3, and physical therapy for 1. Children receiving these services were significantly more likely to have had low developmental scores in the expected domains, when compared to those not receiving services. The majority of developmentally delayed children were not receiving adequate, if any, resource support. Medical and surgical history was not associated with greater likelihood of receipt of services.nnnCONCLUSIONSnChildren with congenitally malformed hearts who are now of school age are at risk for developmental challenges and academic difficulties, yet many do not receive services to optimize performance. Modification of current practice to include systematic, periodic screening, as well as the availability of a resource person for information and referral, may be warranted to meet the ongoing needs of these children and their families, and to optimize their health and well-being.

The Role of Rehabilitation Specialists in Canadian NICUs

Rehabilitation specialists are an integral part of the team in the neonatal intensive care unit (NICU). A national survey was conducted to elucidate the current roles of rehabilitation specialists. Occupational therapy (OT), physical therapy (PT), and speech and language pathology (SLP) departments in all Canadian health care institutions with tertiary level NICUs (n = 38) were surveyed by telephone. Results indicate that 16% have no rehabilitation coverage, while 11% receive very limited external services (< 1/month). Over half of the OT and PT departments provide weekly services whereas only 5/38 provide SLP coverage. Service delivery includes assessment and a number of therapeutic interventions. Splinting and feeding are predominantly performed by OT, whereas chest physiotherapy and ROM are carried out primarily by PT. Rehabilitation specialists are actively involved in education and case management. The extent of involvement of rehabilitation specialists was discrepant, and highly associated with the type of facility. Rehabilitation services, when provided, are comprehensive and include evaluation, treatment, teaching, decision-making, and family support.

Multixnodality Evoked Potential Findings in Infants With Congenital Heart Defects

Evoked potentials are sensitive prognostic tools in young infants at risk for developmental disability. The objective of this prospective study was to determine whether infants with congenital heart defects demonstrate evoked potential abnormalities prior to or following open heart surgery, and to examine the association between these abnormalities and developmental status 1 year following surgery. A consecutive series of newborns (less than 1 month old) and infants (1 month to 2 years old) were recruited. Somatosensory and brain stem auditory evoked potentials were carried out before or after cardiac surgery, or both. One year later, neurologic examination and standardized measures of motor performance and functional independence were carried out. Twenty-seven newborns and 31 infants underwent perioperative somatosensory evoked potential recordings. Results indicate that perioperative somatosensory evoked potential abnormalities were common in newborns (41%) but not in infants (13%) with congenital heart defects. Brainstem conduction times were within normal limits in all subjects; however, 32% presented with mild elevations in hearing thresholds. All newborns with abnormal somatosensory evoked potentials had abnormal neurologic examinations both perioperatively and again 1 year after open heart surgery. Moreover, standardized developmental assessments 1 year following surgery indicate that all newborns with somatosensory evoked potential abnormalities had developmental deficits in one or more domains. Somatosensory evoked potential abnormalities in the perioperative period are common in newborns with congenital heart defects, and are strongly predictive of persistent developmental delay later. (J Child Neurol 1999;14:702-707).

Advanced Neuroimaging Techniques: Their Role in the Development of Future Fetal and Neonatal Neuroprotection

Injury to the developing brain is associated with significant risk for potential lifelong, and wide-ranging neurodevelopmental consequences. Despite major advances in neonatal intensive care in recent decades, truly informed brain-oriented care of the critically ill neonate remains lacking. Consequently, this has hindered the development of preventive neuroprotective interventions, which is in large part due to the inherent difficulties in diagnosis, timing, and the severity of insults. Recent advances in understanding the cellular mechanisms of neonatal brain injury, together with the successful application of cutting-edge neuroimaging techniques, have markedly improved our understanding of the timing and evolution of structural injury to the immature brain, and its functional consequences. Triggered by these important advances, there is intense and renewed interest in the development of brain-oriented therapies, including neuroprotective strategies aimed at circumventing the injurious effects of neonatal brain insults. This article will provide an overview of normal and abnormal brain development, and explore the role of advanced neuroimaging techniques in neuroprotective therapies in the neonatal intensive care unit.