Objective:This study aimed to evaluate school-age neurodevelopmental outcomes among children with single ventricle heart disease who underwent neonatal Norwood operation with regional cerebral perfusion compared to deep hypothermic circulatory arrest. Additionally, we aimed to identify predictors of school-age development, including early developmental measures.

Study design:Patients enrolled in a prospective randomised trial of infants with single ventricle heart disease undergoing the Norwood operation with either regional cerebral perfusion or deep hypothermic circulatory arrest were included. For the same cohort of patients, this study performed neurodevelopmental testing at 5 years and 10 years of age. At 5 years, a comprehensive neuropsychological evaluation was performed. At 10 years, parent report instruments were used to measure participants’ behaviour and executive function.

Results:Forty-one patients at 5 years of age and 33 patients at 10 years of age completed neurodevelopmental evaluation. There were no significant differences in neurodevelopmental scores between the regional cerebral perfusion and deep hypothermic circulatory arrest groups at either 5 or 10 years. At 5 years of age, the average full scale intelligence quotient (IQ) was 93.4 ± SD18.8. The Bayley Scale of Infant Development Psychomotor Developmental Index (r = 0.68, p < .0001) and mental developmental index (r = 0.64, p < .0001) at 1 year positively correlated with the full scale IQ at 5 years.

Conclusions:Neurodevelopment is delayed in patients with single ventricle heart disease. Neurodevelopmental outcomes at school age did not differ based on the perfusion strategy for the Norwood operation. Mental and psychomotor developmental indices at 1 year are predictive of early school-age measures.