Predictors of Early Neurodevelopmental Outcomes for Children with Congenital Heart Disease: A Report from the Cardiac Neurodevelopmental Outcome Collaborative
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Background
Neurodevelopmental impairments are common in children with congenital heart disease. The Cardiac Neurodevelopmental Outcome Collaborative and Pediatric Cardiac Critical Care Consortium registry linkage allows for the analysis of associations between neurodevelopmental, medical, and sociodemographic variables in a large contemporary cohort.
Methods
Children with congenital heart disease who required surgery with cardiopulmonary bypass at <12 months of age and completed a neurodevelopmental assessment between 11-30 months of age from 2019-2022 were included. Multivariable regression modeling was performed to identify differences in Cognitive, Language, and Motor standard scores from the Bayley Scales of Infant and Toddler Development-III/4 based on congenital cardiac diagnosis, clinical risk factors, and social drivers of health.
Results
Primary analyses included 942 assessments from 868 children completed at 25 sites. Across cardiac diagnostic groups, those with genetic diagnoses (n=116 assessments) scored >1 standard deviation lower on all Bayley indices than those without ( P <0.001 for each). For those without genetic diagnoses, there were differences between cardiac diagnostic groups ( P <0.001) in both Cognitive and Motor indices; participants with transposition of the great arteries exhibited the highest scores compared with other cardiac diagnoses. Lower birth weight, male sex, older age at initial surgery, longer hospital length of stay, more cardiac catheterizations, and lower primary caregiver education were independently associated with worse performance in all indices.
Conclusions
Findings from this multicenter cohort demonstrate variation in neurodevelopmental outcomes according to cardiac diagnosis. Regardless of cardiac diagnosis, the presence of a genetic diagnosis is associated with lower neurodevelopmental scores. Heterogeneous outcomes reinforce the importance of surveillance for all infants undergoing heart surgery in the first year of life.
Clinical Perspective
What is new?
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While genetic diagnoses confer the highest risk of developmental delays and disorders in patients with CHD, cardiac diagnosis also impacts early neurodevelopmental outcomes in non-syndromic patients.
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In non-syndromic patients, those with transposition of the great arteries exhibit higher scores on early ND testing than other common CHD subtypes, while those with single ventricle physiology and atrioventricular septal defects exhibit lower scores.
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Older age at surgery and greater number of interventional cardiac catheterizations may represent newly identified risk factors for adverse early ND outcomes in infants with CHD.
What are the clinical implications?
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Data from the CNOC registry emphasizes the importance of ND follow-up for all infants undergoing cardiac surgery, including those with simpler CHD subtypes.
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The improved outcomes we observed in patients with transposition of the great arteries suggest advances in routine clinical management, including early surgery, may have had a neuroprotective influence.
