Long-Term Neurodevelopmental Outcomes After Repair of Cyanotic Congenital Heart Disease

As patient survival improves with the advancements in the care of children with cyanotic congenital heart disease (CHD), the neurodevelopmental (ND) outcomes in these patients require further investigation. Even as surgical and perioperative survival continue to improve, deficits in cognitive, motor, executive, and psychosocial functioning persist into childhood, adolescence, and adulthood. This narrative review synthesizes current evidence on ND outcomes in Tetralogy of Fallot, transposition of the great arteries, hypoplastic left heart syndrome and single-ventricle/Fontan physiology, and mixed cyanotic CHD cohorts. Chronic hypoxemia, impaired fetal cerebral perfusion, perioperative factors, neuroinflammation, and genetic and environmental contributors collectively shape developmental outcomes. Across lesion types, children demonstrate lower intelligence scores, reduced processing speed, executive dysfunction, motor delays, language impairment, and elevated rates of emotional and behavioral difficulties. Magnetic resonance imaging studies reveal persistent abnormalities in brain volumes that correlate strongly with ND outcomes. Risk factors include surgical complexity, prolonged cyanosis, socioeconomic disadvantage, and postoperative instability. These findings exemplify the need for lifelong developmental surveillance, neuropsychological evaluation, and multidisciplinary care pathways. Understanding lesion-specific vulnerabilities may enable earlier intervention and improved functional outcomes for the expanding population of cyanotic CHD survivors.