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To examine the occurrence of developmental impairments in 3-year-old children with varying severity of congenital heart defects (CHD) and to identify predictors associated with developmental impairment in children with severe CHD.Prospective data collected at birth, 6, 18, and 36 months from the Norwegian Mother and Child Cohort Study (MoBa), conducted by the Norwegian Institute of Public Health, were linked with a nationwide medical CHD registry, and 175 three year olds with CHD in a cohort of 44,044 children were identified. Children with mild/moderate (n = 115) and severe (n = 60) CHD were compared with children without CHD (43,929) on motor, communication, and social impairments as reported by mothers in Norwegian Mother and Child Cohort Study questionnaires. Predictors of developmental impairment were analyzed for the group with severe CHD.Children with severe CHD had >3 times higher odds of communication and gross motor impairments compared with controls, and had 2 times higher odds of any developmental impairment compared with controls. Children with mild and moderate CHD had >2 times higher odds of gross motor impairment but did not otherwise differ from controls. Predictors of impairment identified were previous developmental impairments and smaller head circumference at birth.Children with severe CHD have increased odds of developmental impairments at age 3 years. Early developmental impairments are associated with later developmental impairments, suggesting lasting impairments and not merely temporary delay. Patient-specific conditions at birth should be considered and motor and communication support provided to potentially improve outcomes in children with CHD.