Neurodevelopmental impairment remains a major concern in children with congenital heart disease (CHD), especially among those requiring cardiac surgery during infancy. A cross-sectional analysis published in JAMA Network Open evaluated early neurodevelopmental outcomes in children younger than 30 months enrolled in the Cardiac Neurodevelopmental Outcome Collaborative (CNOC) registry. The registry data were linked with the Pediatric Cardiac Critical Care Consortium registry.
The study included children who underwent cardiac surgical repair or palliation requiring cardiopulmonary bypass before 12 months of age. Neurodevelopmental follow-up data were collected from 25 CNOC sites between May 2019 and June 2022.
A total of 942 neurodevelopmental assessments from 868 children were analyzed. The median age at index operation was 20 days. Overall, 56.1% of children were male, and 11.8% had a genetic diagnosis. Neurodevelopmental outcomes were assessed using the cognitive, language, and motor indexes of the Bayley Scales of Infant and Toddler Development, Third and Fourth Editions.
Findings
- Children with genetic diagnoses scored more than 1 standard deviation lower across all Bayley indexes compared with those without genetic diagnoses. Cognitive scores differed by 18.8 points (95% CI, 15.3–22.4), language scores by 16.1 points (95% CI, 12.2–20.0), and motor scores by 20.9 points (95% CI, 18.3–23.5) (P < 0.001 for all).
- After adjustment for other clinical and demographic factors, cardiac diagnosis remained significantly associated with cognitive and motor outcomes. Children with dextro-transposition of the great arteries had the highest adjusted scores.
- Lower cognitive scores were observed in children with coarctation (β, –4.5; 95% CI, –7.8 to –1.2), single-ventricle physiology (β, –4.0; 95% CI, –6.4 to –1.7), and other cardiac diagnoses compared with the reference group.
- Lower birth weight, male sex, older age at initial surgery, longer hospital length of stay, more cardiac catheterizations, and lower primary caregiver educational level were independently associated with worse cognitive, language, and motor performance.
The findings showed substantial variation in early neurodevelopmental outcomes across CHD diagnoses. Regardless of cardiac diagnosis, genetic diagnoses were associated with lower neurodevelopmental scores.