Five-year neurocognitive and health outcomes after the neonatal arterial switch operation

We sought to assess the 5-year neurocognition and health of an interprovincial inception cohort undergoing the arterial switch operation for transposition of the great arteries.

Sixty-nine consecutive neonates had operations from 1996-2003 with full-flow cardiopulmonary bypass and selective deep hypothermic circulatory arrest. Outcomes were recorded at 58 +/- 9 months of age. Univariate and multivariate analyses were used to identify outcome predictors, including surgical subtype and preoperative, operative, and postoperative variables.

There was 1 (1.5%) operative death. Two children were lost to follow-up, and 1 was excluded because of postdischarge meningitis. Outcomes are reported for 65 survivors. Two (3%) children have cerebral palsy, and 7 (11%) have language disorders, 4 of whom also meet the criteria for autism spectrum disorder. Two of the 4 children with autism have an affected older sibling. Of the 61 children without autism, scores approach those of peers, with a full-scale intelligence quotient of 97 +/- 16, a verbal intelligence quotient of 97 +/- 18, a performance intelligence quotient of 96 +/- 15, and a visual-motor integration score of 95 +/- 16. Mother's education, birth gestation or weight, and postoperative plasma lactate values account for 21% to 32% of the variance of these scores. Septostomy adds 7% to the variance of visual-motor integration scores.

Most preschool children do well after surgical correction for transposition of the great arteries, including complex forms. Potentially modifiable variables include high preoperative plasma lactate levels and septostomy. A minority of children were given diagnoses of language disorders, including autism, in which familial factors likely contribute to outcome.