Long-term neurodevelopmental outcomes in school-aged children after neonatal arterial switch operation

Objective: Neurodevelopmental status of children between 8 and 14 years of age after neonatal arterial switch operation for transposition of the great arteries has not previously been systematically evaluated. Methods: Within a longitudinal study, 60 unselected children operated on as neonates with combined deep hypothermic circulatory arrest and low-flow cardiopulmonary bypass were reevaluated at the age of 7.9 to 14.3 years (mean +/- SD 10.5 +/- 1.6 years). Clinical neurologic status and standardized tests to assess gross motor function, intelligence, acquired abilities, language, and speech were carried out, and the results were related to preoperative, perioperative, and postoperative status, to management, and to neurodevelopmental status at a mean age of 5.4 years. Results: Neurologic and speech impairments were evidently more frequent (27% and 40%, respectively) than in the general population. Intelligence and socioeconomic status were not different (P = .29 and P = .11), whereas motor function, acquired abilities, and language were reduced (P less than or equal to .04 for each). Overall rate of developmental impairment in one or more domains was 55%, compared with 26% at age 5.4 years. Multivariable analysis showed that severe preoperative acidosis and hypoxia. predicted reduced motor function (mean deficit 52.7 points, P < .001), whereas longer bypass duration predicted both neurologic (odds ratio per 10 minutes of bypass duration 1.8, P = .04) and speech (odds ratio per 10 minutes of bypass duration 1.9, P = .02) dysfunction, and perioperative and postoperative cardiocirculatory insufficiency predicted neurologic (odds ratio 6.5, P = .04) and motor (mean deficit 6.8 points, P = .03) dysfunction. Conclusions: The neonatal arterial switch operation with combined circulatory arrest and low-flow bypass is associated increasingly with age, with reduced neurodevelopmental outcome but not with cognitive dysfunction. In our experience, the risk of long-term neurodevelopmental impairment after neonatal corrective cardiac surgery is related to deleterious effects of the global perioperative management and to special adverse effects of prolonged bypass duration. Severe preoperative acidosis and hypoxia and postoperative hemodynamic instability must be considered as important additional risk factors.