Abstract

Advances in prenatal diagnosis, perioperative management, and postoperative care have dramatically increased the population of survivors of neonatal and infant heart surgery. The high survival rate of these patients into adulthood has exposed the alarming prevalence of long-term neuropsychological and psychiatric morbidities. Dextro-transposition of the great arteries (d-TGA) is one of the most extensively studied cyanotic congenital heart defect (CHD) with regard to neurodevelopmental outcomes. Landmark studies have described a common neurodevelopmental and behavioral phenotype associated with d-TGA. Children with d-TGA display impairments in key neurocognitive areas, including visual–spatial and fine motor abilities, executive functioning, processing speed, and social cognition. As they grow older, they may face additional challenges with a worsening of deficits in higher order cognitive skills, problems in psychosocial adjustment and a higher-than-expected rate of psychiatric disorders, such as attention-deficit hyperactivity disorder, depression, and anxiety. The aim of this review is to summarize the available recent data on neuropsychological and psychiatric outcomes in individuals with d-TGA after the arterial switch operation. We present findings within a life-span perspective, with a particular emphasis on the emerging literature on adolescent and young adult outcomes. Finally, we propose avenues for future research in the CHD adult neuropsychology field. Among these avenues, we explore the potential mechanisms by which pediatric neurodevelopmental impairments may have lifelong adverse effects as well as alternative interventions that could optimize outcomes.

Highlights

  • Dextro-transposition of the great arteries (d-TGA) accounts for 5–7% of congenital heart defects (CHD), with a prevalence of 0.2 per 1,000 live births [1, 2]

  • Over the last two decades, dramatic progress was made in the understanding of neuropsychological and psychiatric outcomes of individuals with d-TGA

  • All the authors (LK, DB, MM, DK, NG, DCB, and JC) approved the final version of the manuscript and agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved

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Summary

Introduction

Dextro-transposition of the great arteries (d-TGA) accounts for 5–7% of congenital heart defects (CHD), with a prevalence of 0.2 per 1,000 live births [1, 2]. Individuals with d-TGA represent a unique and relatively homogeneous study cohort with the Arterial Switch Operation (ASO) being the standard-of-care. D-TGA is infrequently associated with extra-cardiac anomalies, including genetic abnormalities, reducing potential confounding variables in follow-up studies. Long-term outcomes in d-TGA, and in CHD population as a whole, pose a public health challenge for screening, diagnosis, and treatment. The aim of this state-of-the-art review is to integrate recent data on neuropsychological and psychiatric outcomes in d-TGA after the ASO within a life-span perspective. We propose avenues for future research, including discussion on the potential mechanisms of long-term impairments and interventions to optimize outcomes

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