Adolescents With d-Transposition of the Great Arteries Corrected With the Arterial Switch Procedure: Neuropsychological Assessment and Structural Brain Imaging

Study Questions:

What are the neurodevelopmental outcomes and brain imaging findings of adolescents who underwent the arterial switch procedure for d-transposition of the great arteries (d-TGA) in the neonatal period?


Patients were initially enrolled in the Boston Circulatory Arrest Study at the time of their arterial switch, randomizing patients to deep hypothermia with either circulatory arrest or continuous low-flow bypass. Subjects underwent assessment of academic achievement, memory, executive functions, visual-spatial skills, attention, and social cognition. Brain magnetic resonance imaging (MRI) was performed as well. A referent group of adolescents was recruited to undergo brain MRI, given the absence of a national representative standardization sample.


Of 159 eligible adolescents, 139 participated. Few significant differences were seen between the bypass techniques. The scores of patients in the treatment groups tended to be lower than those of the normative populations for individual instruments. A significant portion (65%) of subjects had received remedial academic or behavioral services. Brain MRI abnormalities were more common in patients with d-TGA (33%), as compared with controls (4%). The Connors ADHD index differed significantly from the expected population mean of 50 ± 10 (p = 0.001), with 19% of adolescents having scores of ≥65 suggestive of clinically significant attention issues.


The authors concluded that adolescents with d-TGA following the arterial switch operations are at increased neurodevelopmental risk, and may benefit from ongoing neurodevelopmental surveillance and assessment.


The arterial switch procedure is now performed with minimal mortality and generally excellent hemodynamic results. Previous studies have raised concern for the potential for adverse neurodevelopmental outcomes in very young children, as well as high rates of abnormal brain imaging in the perioperative period. This is the first study to extend the follow-up into adolescence. There were few significant differences in neurodevelopmental outcomes between those patients whose bypass technique consisted of deep hypothermia with continuous low-flow bypass, as compared with deep hypothermia with circulatory arrest. These results suggest that patients following the arterial switch operation should have continued close monitoring of their neurodevelopmental status through adolescence.

Clinical Topics: Congenital Heart Disease and Pediatric Cardiology, Noninvasive Imaging, Congenital Heart Disease, CHD and Pediatrics and Imaging, Magnetic Resonance Imaging

Keywords: Neuropsychological Tests, Hypothermia, Boston, Transposition of Great Vessels, Magnetic Resonance Imaging, Brain

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