Comparing Parent Perception of Neurodevelopment after Primary versus Staged Repair of Neonatal Symptomatic Tetralogy of Fallot.

Objective: To assess the association between primary and staged repair of neonatal symptomatic tetralogy of Fallot (sTOF) and neurodevelopmental outcomes in preschool through school-age children.

Study Design: Multicenter cohort (n = 9 sites) study of patients with sTOF who underwent neonatal intervention between 2005 and 2017. The neurodevelopmental outcomes measures included caregivers' ratings of executive function with the Behavior Rating Inventory of Executive Function, and psychosocial functioning with the Behavior Assessment System for Children - third Edition (BASC-3). Results were compared with normative data and by treatment strategy (primary repair vs staged repair). A parent survey assessed history of disabilities and access to services related to neurodevelopment.

Results: Although the majority of patients (median age 8.3 years, IQR 5.7-11.2) had median Behavior Rating Inventory of Executive Function and BASC-3 scores within the normal range, a proportion had clinically elevated (abnormal) scores, especially in the school-age patient subgroup (Behavior Rating Inventory of Executive Function 24%-30% and BASC 20%-37%). There were no statistically significant differences based on treatment strategy for either the Behavior Rating Inventory of Executive Function or BASC-3. However, lower birth weight, genetic syndrome, and medical complexity were significantly associated with worse executive function, and lower maternal education was associated in school-age children with lower executive and psychosocial functioning. Ongoing disabilities were relatively common (learning disability 35%, speech delay 33%, developmental delay 31%), although up to 50% of children were not receiving educational or developmental services.

Conclusions: Elevated executive and psychosocial concerns are present in the patient population with sTOF. Although initial treatment strategy appears unrelated to neurodevelopmental outcomes, lower birth weight, genetic syndrome, and medical complexity and lower maternal education are risk factors. Early recognition of neurodevelopmental concerns can facilitate access to appropriate neurodevelopmental services in this high-risk group.