Risk and Prevalence of Developmental Delay in Young Children With Congenital Heart Disease

Abstract

WHAT'S KNOWN ON THIS SUBJECT: Children with congenital heart disease demonstrate a high prevalence of low-severity developmental problems in the areas of language, motor skills, attention, and executive function. Systematic evaluation has been recommended to promote early detection of problems and ensure appropriate intervention. WHAT THIS STUDY ADDS:This study presents results of longitudinal testing in early childhood. Developmental delays were common. Feeding difficulty and medical and genetic comorbidities increased risk for delays. Exposure to risk and prevalence of delay change over time; therefore, repeated evaluations are warranted.abstract BACKGROUND AND OBJECTIVE: Children with congenital heart disease (CHD) are at risk for developmental delay (DD). Changes in cognitive, language, and motor skills in early childhood have not been described. We report the results of a structured approach using longitudinal testing to identify problems and ensure early intervention in accordance with published guidelines.METHODS: Bayley Scales of Infant Development, Third Edition, were used to assess cognitive, language, and motor skills in 99 children with CHD. Subjects were evaluated 3 to 6 times in the first 3 years of life. DD was defined as scores .1 SD below the population mean. RESULTS:Cardiac anatomy was single ventricle (1V) in 34 subjects and 2 ventricles (2V) in 65. Medical comorbidities were present in 21% and genetic syndromes in 19%. Most subjects (75%) had DD in $1 area at $1 assessments. Subjects with 1V anatomy had equivalent outcomes to those with 2V. Cognitive and language scores declined in subjects with genetic syndromes but were stable and within the average range for subjects with 1V and 2V. Motor scores improved for subjects with 1V and 2V but remained low for those with genetic syndromes. In addition to age, need for supplemental tube feeding, longer cardiopulmonary bypass time, and shorter time since last hospitalization were significant predictors of developmental outcomes.CONCLUSIONS: DDs in young children with CHD are both common and dynamic. Providers should encourage longitudinal surveillance for children with CHD because exposure to risk and prevalence of DD change over time. Pediatrics 2014;133:e570-e577 AUTHORS:

Perioperative cerebral hemodynamics and oxygen metabolism in neonates with single-ventricle physiology

“…However, despite this increased survival, a high risk for neurodevelopmental co-morbidity persists [2]. Neurodevelopmental outcomes are characterized by a diverse spectrum of developmental delays and disabilities including impaired executive functions with the prevalence and severity increasing with the complexity of the CHD [3][4][5][6][7]. The etiology of these neurodevelopmental disorders is complex and several variables including pre-existing brain abnormalities and evolving factors in the perioperative period are believed to play a significant role [8][9][10][11].…”

Implementation of Developmental Screening Guidelines for Children with Congenital Heart Disease

“…[13][14][15][16][17] In the postnatal period, hypoxia, decreased cardiac output or altered circulation patterns, exposure to cardiopulmonary bypass and circulatory arrest, risk of stroke, polycythemia, and prolonged hospitalizations can contribute to the neurodevelopmental morbidity seen in these children. 4,5,7,8,18,19 In addition, genetic vulnerabilities frequently coexist with CHD, such as the association with the Apolipoprotein E є2 genotype. 20 Early research into improving neurodevelopmental outcomes in children with CHD focused on modifiable perioperative risk factors.…”

“…There is significant variability in the degree of developmental impairments, which include impairments in fine and gross motor skills, visual-spatial skills, executive functioning, social cognition, and memory, all of which can affect overall academic achievement and longterm quality of life. [3][4][5][6][7][8][9][10][11] The reasons that children with CHD are at risk for neurodevelopmental problems are multifactorial and include genetic factors, as well as prenatal and postnatal physiology and deleterious postnatal exposures. 5,8,12 Preoperative brain imaging studies in patients with cardiac defects characterized by altered cerebral blood flow and oxygen content reveal reduced brain volume and delayed brain maturation, which increases susceptibility to white matter injury and is reminiscent of the encephalopathy of prematurity.…”

“…[3][4][5][6][7][8][9][10][11] The reasons that children with CHD are at risk for neurodevelopmental problems are multifactorial and include genetic factors, as well as prenatal and postnatal physiology and deleterious postnatal exposures. 5,8,12 Preoperative brain imaging studies in patients with cardiac defects characterized by altered cerebral blood flow and oxygen content reveal reduced brain volume and delayed brain maturation, which increases susceptibility to white matter injury and is reminiscent of the encephalopathy of prematurity. [13][14][15][16][17] In the postnatal period, hypoxia, decreased cardiac output or altered circulation patterns, exposure to cardiopulmonary bypass and circulatory arrest, risk of stroke, polycythemia, and prolonged hospitalizations can contribute to the neurodevelopmental morbidity seen in these children.…”

Hyperoxia and the Immature Brain

“…Several outcome studies of patients with congenital heart disease showed that, in early childhood, motor development seems to be more affected than cognitive development [137,138] . This seems to change when the child gets older, with improvements in motor scores, behavioural problems, and learning difficulties [139] . Data on the long-term consequences of restrictive oxygen supplementation until school age is lacking.…”