Childhood Neurocognitive Outcomes in Patients With Fontan Circulation

Area of Science:

• Pediatric Cardiology
• Neurodevelopmental Pediatrics
• Congenital Heart Disease

Background:

• The Fontan procedure is a palliative surgery for single-ventricle congenital heart defects.
• Neurocognitive outcomes in children with Fontan circulation require ongoing assessment.
• Identifying risk factors for neurodevelopmental deficits is crucial for early intervention.

Purpose of the Study:

• To evaluate neurocognitive outcomes in a modern cohort of patients with Fontan circulation.
• To identify factors influencing neurodevelopmental risk in this population.

Main Methods:

• Retrospective cohort study of single-ventricle patients (born 2004-2019) undergoing staged univentricular palliation.
• Standardized neurocognitive testing using Wechsler intelligence scales at approximately 5 years of age.
• Analysis of demographic, surgical, and perioperative data to determine neurodevelopmental risk factors.

Main Results:

• 204 patients were analyzed, with a mean Full-Scale IQ (FSIQ) of 93.5±12.3, below population norms.
• 4% had severe cognitive impairment (FSIQ <70) and 20.5% had moderate impairment (FSIQ 70-84).
• Microcephaly, lower parental education, preoperative complications, and longer postoperative stay were linked to lower FSIQ; head circumference Z-score, higher parental education, and shorter hospital stay correlated with higher FSIQ.

Conclusions:

• Contemporary Fontan patients exhibit low-normal mean FSIQ with a significant proportion experiencing intellectual impairment.
• Hypoplastic left heart syndrome and cardiopulmonary bypass use during neonatal palliation did not correlate with lower FSIQ.
• Preoperative condition and patient-specific factors are more influential on cognitive function than surgical palliation variables.