Neuropsychological and Psychiatric Outcomes in Dextro-Transposition of the Great Arteries across the Lifespan: A State-of-the-Art Review

Kasmi, Leïla; Bonnet, Damien; Montreuil, Michèle; Kalfa, David; Geronikola, Nikoletta; Bellinger, David C.; Calderon, Johanna · 2017 · OpenAlex-citations

DOI: 10.3389/fped.2017.00059

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Summary

This state-of-the-art review examines the long-term neuropsychological and psychiatric outcomes of individuals with dextro-transposition of the great arteries (d-TGA) following the arterial switch operation (ASO). Motivated by increased survival rates into adulthood, the authors address the growing public health challenge of managing chronic neurodevelopmental and mental health morbidities in this population. The review synthesizes existing literature to characterize the cognitive and behavioral phenotype of d-TGA patients across the lifespan, from infancy through adulthood, and proposes mechanisms for these impairments and avenues for future intervention. The authors conducted a comprehensive narrative review of studies focusing on d-TGA survivors, with particular emphasis on longitudinal data from the Boston Circulatory Arrest Study (BCAS) and the Aachen Study. The analysis covers neuropsychological assessments in children, adolescents, and adults, as well as psychiatric diagnoses and psychosocial functioning. The review highlights that d-TGA provides a relatively homogeneous cohort for study due to the standardization of the ASO and low prevalence of extra-cardiac anomalies. Findings indicate that while full-scale IQ scores in children with d-TGA are generally within the normal range, specific neurocognitive deficits are prevalent. These include impairments in visual–spatial skills, fine motor abilities, executive functioning (particularly planning, cognitive flexibility, and working memory), processing speed, and social cognition, such as Theory of Mind deficits. These deficits persist into adolescence, where patients exhibit lower scores in academic skills, memory, and executive functions compared to normative means. Psychiatric outcomes in adolescents reveal a higher-than-expected prevalence of attention-deficit hyperactivity disorder (ADHD), depression, anxiety, and post-traumatic stress symptoms. Approximately 15% of adolescents fall within the pathological range for psychosocial functioning. In adults, data are scarcer, but available studies suggest elevated risks for cognitive impairments in processing speed and executive attention, as well as higher rates of mood and anxiety disorders compared to the general population. Early cognitive deficits, particularly in executive function, are identified as strong predictors of later academic underachievement and psychosocial difficulties. The review concludes that d-TGA survivors face a distinct neurodevelopmental signature that requires lifelong monitoring. The authors emphasize that early cognitive impairments can cascade into more severe dysfunction as age-related expectations increase, affecting social relationships and employability. They advocate for improved screening, targeted interventions to optimize executive function and social cognition, and facilitated access to mental health services during the transition from pediatric to adult care. Future research is needed to elucidate the mechanisms underlying these lifelong impairments and to develop effective strategies for mitigating adverse outcomes in this growing adult population.

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• cognitive impairment