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J Thorac Cardiovasc Surg 2008;136:1413-1421
© 2008 The American Association for Thoracic Surgery

Congenital Heart Disease

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

^a Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada
^b Pediatric Rehabilitation Outcomes Evaluation and Research, Glenrose Rehabilitation Hospital, Edmonton, Alberta, Canada
^c Department of Pediatrics, University of Manitoba, Winnipeg, Manitoba, Canada
^d School of Public Health, University of Alberta, Edmonton, Alberta, Canada
^e Department of Pediatrics, University of Calgary, Calgary, Alberta, Canada
^f Department of Surgery, University of Alberta, Edmonton, Alberta, Canada

Received for publication January 8, 2008; revisions received March 28, 2008; accepted for publication May 4, 2008.

^_* Address for correspondence: Charlene M. T. Robertson, MD, Room 242, GlenEast, Glenrose Rehabilitation Hospital, 10230–111 Avenue, Edmonton, Alberta T5G 0B7, Canada. (Email: charlene.robertson{at}capitalhealth.ca).

Objectives: 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.

Methods: 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.

Results: 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.

Conclusions: 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.