To describe developmental and functional outcomes of children with congenital heart defects (CHDs) at school entry after open heart surgery. Infants with CHDs who underwent surgical repair in infancy were recruited and assessed prospectively for developmental progress. At 5 years of age (64.2 +/- 11.3 months), 94 subjects were evaluated in a blind fashion by using a variety of standardized measures. Mean IQ scores were in the low average range (90-94). Receptive language was in the average range (103.6 +/- 14.4). Behavioral difficulties were common (27.1%), with internalizing problems being more frequent. Functional limitations in socialization (93.0 +/- 17.1), daily living skills (94.6 +/- 16.4), communication (90.0 +/- 14.1), and adaptive behavior (92.1 +/- 15.8) were noted in 11% to 17% of children. With the Functional Independence Measure for Children, 20% to 22% of subjects were more dependent than their peers in self-care and social cognition, although few (4.5%) had mobility restrictions. Predictors of developmental and functional limitations included: abnormal postoperative neurologic examination, microcephaly, deep hypothermic circulatory arrest time, palliation, acyanotic heart lesion, age at surgery, and maternal education. After infant open-heart surgery, children with CHDs may exhibit a range of developmental difficulties at school entry that enhances risk for learning challenges and decreased social participation.