Congenital heart disease (CHD) is the leading cause of fetal malformations and remains a major determinant of morbidity in childhood. Children with CHD are at increased risk of neurodevelopmental disorders (NDD). The severity of CHD strongly influences clinical management and long-term outcomes, including neurodevelopment. Adverse maternal environment may play a major role in CHD and NDD induction. Additionally, contribution of CHD severity to NDD remains unclear.

We analyzed obstetrical and pediatric data from a French multicenter retrospective cohort including 334 women who delivered infants with CHD between 2012 and 2023. Neurodevelopmental follow-up at 2, 5 or 7 years was available for a third of them. CHD severity was categorized as critical-complex, non-critical complex and non-complex according to the classification used by the French Reference Center for Complex Congenital Cardiac Malformations (M3C) and time to cardiac surgery. NDD were assessed using the Strengths and Difficulties Questionnaire (SDQ), the Ages and Stages Questionnaire (ASQ) and a standardized teacher-completed questionnaire. Multinomial multivariate logistic regression was used to evaluate associations between maternal environmental factors, CHD severity and NDD.

Maternal psychological distress (OR 0.39, 95% CI [0.18–0.87]) and nulliparity (OR 1.77, 95% CI [1.08–2.90]) were associated with a lower probability of severe CHD. CHD severity was not significantly associated with NDD at 2, 5 or 7 years. Maternal age < 25 years or > 30 years, second parity and gestational weight gain < 12 kg were associated with a higher risk of combined CHD and NDD.

Our study is the first to demonstrate the associations between specific maternal environmental risk factors and NDD in CHD infants when considering CHD severity. We showed no difference in NDD occurrence regarding CHD severity. These results support systematic neurodevelopmental screening in all CHD patients during childhood regardless the severity of the cardiac pathology.