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

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.

Attention-deficit/hyperactivity disorder symptoms in children with surgically corrected Ventricular Septal Defect, Transposition of the Great Arteries, and Tetralogy of Fallot

“…The different pattern of comorbidity in children with CHD and attention-deficit/hyperactivity disorder symptoms could implicate that the pathogenesis of their attention-deficit/hyperactivity disorder is different from children with idiopathic attention-deficit/hyperactivity disorder. 10,32 The marked discrepancy between parents and teachers' rating was seen both in children with CHD and among controls, although the difference was less obvious in controls due to the overall lower attention-deficit/hyperactivity disorder symptoms scores. It is evident that levels of attention-deficit/hyperactivity disorder symptoms perceived by parents are above the levels by teachers as previously reported in both children with idiopathic attentiondeficit/hyperactivity disorder 19,33 and in children with CHD with attention-deficit/hyperactivity disorder.…”

‘Big issues’ in neurodevelopment for children and adults with congenital heart disease

“…Up to 50% of children requiring cardiac intervention exhibit NDD, including mild cognitive impairments, difficulties with attention and hyperactivity, deficits in motor functioning, social interaction, language and communication skills, and delayed executive function,2–7 which may persist into school age and beyond 8–10. As expected, NDD has a detrimental effect on educational achievement and attainment, which consequently affects later employability, independence and relationships, and may heighten the burden of psychological disturbance and reduce overall QOL11–13 (figure 1).…”

A referral pathway for potentially abnormal neurodevelopment in children with heart disease in the United Kingdom: a Delphi consensus

“…Neurodevelopmental difficulties can potentially be the most devastating long-term sequelae for children with CHD [28][29][30] and as the number of survivors with complex cardiac conditions increase, there is a growing demand for these children to have adequate support within the wider healthcare system. Routine formal neurodevelopmental testing can be difficult to enforce within the constraints of the NHS.…”