Childhood Neurocognitive Outcomes in Patients With Fontan Circulation

To assess childhood neurocognitive outcomes in a contemporary Fontan cohort and to identify neurodevelopmental risk factors.

This retrospective cohort study enrolled patients with a single ventricle born between 2004 and 2019 receiving standardized neurocognitive testing with age-appropriate Wechsler intelligence scales after staged univentricular palliation. Demographic, surgical, and perioperative data were collected to evaluate neurodevelopmental risk factors.

Data from 204 patients assessed at 5.0 (interquartile range, 4.5-6.1) years of age were analyzed. Full-scale IQ (FSIQ) was in the lower normal range (93.5±12.3) but below population norms (P<0.001). Severe cognitive impairment (FSIQ <70) was noted in 4% and moderate impairment (FSIQ 84-70) in 20.5%. FSIQ was not different between hypoplastic left heart syndrome (n=112) and other univentricular heart defects (93.7±12.6 versus 93.3±11.9, P=0.821). Use of cardiopulmonary bypass for neonatal palliation (n=142) was not related to lower FSIQ (93.7±12.8 versus 92.9±11.0, P=0.670). Microcephaly, lower parental education level, and complications before and length of postoperative stay after neonatal palliation were independently associated with FSIQ below average. Head circumference Z-score, higher parental education, and shorter cumulative hospital stay were related with favorable outcome (FSIQ>100).

In this contemporary Fontan cohort, mean FSIQ was in the low normal range but with a higher proportion of patients with intellectual impairment. Hypoplastic left heart syndrome or use of cardiopulmonary bypass for neonatal palliation was not associated with lower FSIQ scores. Preoperative condition and patient-related factors were more important determinants of cognitive function than variables related to surgical palliation.