Childhood Neurocognitive Outcomes in Patients With Fontan Circulation.

BACKGROUND

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

METHODS

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.

RESULTS

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).

CONCLUSIONS

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.