Background and Objectives Mental health outcomes for survivors of critical congenital heart disease (CHD) remain under-investigated. We sought to examine psychiatric disorders and psychosocial functioning in adolescents with single ventricle CHD and to explore whether patient-related risk factors predict dysfunction. Methods This cohort study recruited 156 adolescents with single ventricle CHD who underwent the Fontan procedure and 111 healthy referents. Participants underwent comprehensive psychiatric evaluation including a clinician-rated psychiatric interview and parent- and self-report ratings of anxiety, disruptive behavior – including attention deficit hyperactivity disorder (ADHD), and depressive symptoms. Risk factors for dysfunction included IQ, medical characteristics, and concurrent brain abnormalities. Results Adolescents with single ventricle CHD had higher rates of lifetime psychiatric diagnosis compared with referents (CHD: 65%, referent: 22%; P<0.001). Specifically, they had higher rates of lifetime anxiety disorder and ADHD diagnosis (P<0.001 each). The CHD group scored lower on the primary psychosocial functioning measure, the Children’s Global Assessment Scale (CGAS), than referents (CHD median [interquartile range]: 62 [54–66], referent: 85 [73–90]; P<0.001). The CHD group scored worse on measures of anxiety, disruptive behavior, and depressive symptoms. Genetic comorbidity did not impact most psychiatric outcomes. Risk factors for anxiety disorder, ADHD, and lower CGAS scores included lower birth weight, longer duration of deep hypothermic circulatory arrest, lower intellectual functioning, and male gender. Conclusions Adolescents with single ventricle CHD display a strikingly high risk of psychiatric morbidity, particularly anxiety disorders and ADHD. Early identification of psychiatric symptoms is a key component of the management of patients with CHD.

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TGA Transposition of the great arteries ToM Theory of mindAIM Cardiac malformations resulting in cyanosis, such as transposition of the great arteries (TGA), have been associated with neurodevelopmental dysfunction. The purpose of this study was to assess, for the first time, theory of mind (ToM), which is a key component of social cognition and executive functions in school-aged children with TGA.METHOD Twenty-one children (14 males, seven females; mean age 7y 4mo; SD 3mo) who underwent neonatal open-heart surgery for TGA using full-flow cardiopulmonary bypass were compared with 21 typically developing age-matched children (12 males, nine females; mean age 7y 6mo; SD 3.8mo) using different neuropsychological measures specifically designed to assess executive function (cognitive and response inhibition, verbal and spatial working memory, and planning). They were also given two ToM tasks (first-and second-order false belief understanding).RESULTS General IQ was within the normal range in both the TGA group and the comparison group (mean IQ 113 [SD 9.3] and 118 [SD 10.1] respectively), but performance on all executive functions and on ToM (first and second level) was significantly lower in the TGA group (p values of 0.02, 0.01, and 0.004 respectively). A discriminant multivariate analysis provided evidence for cognitive and behavioural inhibition as well as performance on false belief tasks as being the most important contributors to the differentiation between the groups (p=0.03).INTERPRETATION Children with TGA demonstrate great difficulties in exerting cognitive and behavioural inhibition. They also present specific deficits in false belief understanding, which were related to immature executive abilities.

It is widely recognised that children with congenital heart disease (CHD) are at high risk for neurodevelopmental impairments including attention deficit hyperactivity disorder and autism spectrum disorder symptoms. Executive function impairments are one of the most prominent neurodevelopmental features associated with CHD. These deficits can have widespread debilitating repercussions in children's neurocognitive, behavioural, and psycho-social development. There is a crucial gap in research regarding the efficacy of preventive or treatment strategies for these important cognitive morbidities. Executive functions are complex neurocognitive skills highly amenable to improvement. Evidence-based interventions have shown promising results in other paediatric populations, strongly suggesting that they might also benefit the growing population of children with CHD. In this review, we summarise the available data on executive function impairments in children and adolescents with CHD. We underline the important co-morbidity of executive dysfunction with other cognitive and psychiatric issues in CHD, which raises awareness of the crucial need to prevent or at least mitigate these deficits. Finally, we summarise future avenues for research in terms of interventions that may help reduce executive function impairments in youth with CHD.

Background: Neurodevelopmental impairment is common in children with congenital heart disease (CHD), yet postnatal variables explain only 30% of the variance in outcomes. To explore whether the antecedents for neurodevelopmental disabilities might begin in utero , we analyzed whether fetal brain volume predicted subsequent neurodevelopmental outcome in children with CHD. Methods: Fetuses with isolated CHD and sociodemographically comparable healthy control fetuses underwent fetal brain MRI and 2-year neurodevelopmental evaluation with the Bayley Scales of Infant and Toddler Development (Bayley-III) and the Adaptive Behavior Assessment System (ABAS-3). Hierarchical regression evaluated potential predictors of Bayley-III and ABAS-3 outcomes in the CHD group, including fetal total brain volume adjusted for gestational age and sex, sociodemographic characteristics, birth parameters, and medical history. Results: The CHD group (n=52) had lower Bayley-III cognitive, language, and motor scores than the control group (n=26), but fetal brain volumes were similar. Within the CHD group, larger fetal total brain volume correlated with higher Bayley-III cognitive, language, and motor scores, and ABAS-3 adaptive functioning scores (r=0.32-0.47; all P<0.05), but not in the control group. Fetal brain volume predicted 10 21% of the variance in neurodevelopmental outcome measures in univariate analyses. Multivariable models that also included social class and postnatal factors explained 18-45% of the variance in outcome, depending on developmental domain. Moreover, in final multivariable models, fetal brain volume was the most consistent predictor of neurodevelopmental outcome across domains. Conclusions: Small fetal brain volume is a strong independent predictor of 2-year neurodevelopmental outcomes and may be an important imaging biomarker of future neurodevelopmental risk in CHD. Future studies are needed to support this hypothesis. Our findings support inclusion of fetal brain volume in risk stratification models and as a possible outcome in fetal neuroprotective intervention studies.

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This longitudinal study investigates executive functions (EF) in children with transposition of the great arteries (TGA) compared to typically developing children at a key age period between 5 and 7 years. We explored the presence and evolution of specific impairments on three core EF components (inhibition, working memory, and cognitive flexibility). Ninety children were evaluated for three consecutive years. Results demonstrated significant delays in inhibition and cognitive flexibility despite normal working memory. Impairments did not systematically worsen with age. EF impairments after TGA are dynamic and may affect selective components. Cyanotic congenital heart disease is associated with altered EF development.