No population-based prospective cohort study has assessed the neurodevelopmental outcomes of school-aged children with CHD.

To assess whether some groups of CHDs, more specifically cyanotic CHD (CCHD) with or without heart failure and non CCHD with heart failure, are at greater risk of adverse neurodevelopmental outcomes at 8 years of age.

We used data from the prospective population-based cohort study EPICARD, which included all children with a CHD diagnosed in the prenatal period and up to 1 year of age, between 2005 and 2008 in the greater Paris areas. We looked at the overall intellectual quotient (IQ), and the specific neurocognitive domains. We classified the CHDs based on pathophysiological and clinical management characteristics and compared the outcomes across six groups of CHDs as compared with the control group (minor ventricular septal defects). In order to do so, we used multivariable linear regression models with the Heckman method to account for selection bias due to differential loss to follow-up.

From the 1196 eligible patients for the 8-year follow up, 473 (39.5%) completed the neurodevelopmental evaluations. Compared to the control group, children with CCHD with or without heart failure had lower IQ scores, −8.0 (P<0.01) and −7.3 (P=0.02), respectively. Those with CCHD and non CCHD with heart failure also had lower scores at the language subtest, −1.9 (P<0.01) and − 1.4 (P<0.01), respectively. CCHD and non CCHD with heart failure and CHD with increased pulmonary blood flow had lower learning and memory scores, −1.36 (P<0.01), −1,2 (P=0.05) and − 0.9 (P=0.04), respectively. CCHD with heart failure had lower scores in the executive functions (inhibition) domain, −1.2 (P=0.05)

Children with CHD associated with cyanosis and/or heart failure had significantly lower neurodevelopment scores at 8 years of age, both for overall IQ and specific neurocognitive domains.