CONTEXT Cognitive function and executive function (EF) impairments contribute to the long-term burden of congenital heart disease (CHD). However, the degree and profile of impairments are insufficiently described. OBJECTIVE To systematically review and meta-analyze the evidence on cognitive function and EF outcomes in school-aged children operated for CHD and identify the risk factors for an unfavorable outcome. DATA SOURCES Cochrane, Embase, Medline, and PsycINFO. STUDY SELECTION Original peer-reviewed studies reporting cognitive or EF outcome in 5- to 17-year old children with CHD after cardiopulmonary bypass surgery. DATA EXTRACTION Results of IQ and EF assessments were extracted, and estimates were transformed to means and SE. Standardized mean differences were calculated for comparison with healthy controls. RESULTS Among 74 studies (3645 children with CHD) reporting total IQ, the summary estimate was 96.03 (95% confidence interval: 94.91 to 97.14). Hypoplastic left heart syndrome and univentricular CHD cohorts performed significantly worse than atrial and ventricular septum defect cohorts (P = .0003; P = .027). An older age at assessment was associated with lower IQ scores in cohorts with transposition of the great arteries (P = .014). Among 13 studies (774 children with CHD) reporting EF compared with controls, the standardized mean difference was −0.56 (95% confidence interval: −0.65 to −0.46) with no predilection for a specific EF domain or age effect. LIMITATIONS Heterogeneity between studies was large. CONCLUSIONS Intellectual impairments in CHD are frequent, with severity and trajectory depending on the CHD subtype. EF performance is poorer in children with CHD without a specific EF profile. The heterogeneity in studied populations and applied assessments is large. A uniform testing guideline is urgently needed.

Aim To examine how the ongoing COVID‐19 pandemic impacts child well‐being and family functioning, particularly among children at risk for neurodevelopmental impairments. Methods Families of 73 typically developing children, 54 children born very preterm (VPT) and 73 children with congenital heart disease (CHD) from two prospective cohort studies were assessed prior to (mean age: 10.4 [SD: 1.2] years) and during (mean age: 12.8 [SD: 2.0] years) the pandemic, more specifically, in April/May 2020. Child well‐being and family functioning were assessed with validated, parent‐reported questionnaires and tested with linear mixed models. Group comparison of child distress and parental concerns related to medical implications of COVID‐19 and homeschooling, assessed with 5‐point Likert scales, was done with Mann–Whitney U tests. Results Children's psychological well‐being and family functioning (both, p < 0.001) decreased significantly during the pandemic, irrespective of group. Children with CHD were reported to be more concerned about becoming infected with SARS‐CoV‐2 than were others. Child distress due to homeschooling and parents' concerns about children's academic achievements were significantly higher in VPT and CHD children than in typically developing peers (all p < 0.001). Conclusion The COVID‐19 pandemic substantially impacts the whole family and leads to additional distress in families with children at risk for neurodevelopmental impairments. These families should receive individualised counselling and assistance from healthcare providers and schools during the pandemic.

HighlightsPatients with congenital heart disease (CHD) are at risk for lower working memory.CHD patients showed widespread alterations in white matter microstructure using DTI.Alterations in frontal white matter were associated with working memory problems.This association was independent of CHD type, IQ and group.

Children with congenital heart disease (CHD) may be at increased risk for neurodevelopmental impairments. Long-term behavioral profiles and respective risk factors are less frequently described. The aim of this study was to evaluate multidimensional behavioral outcomes and associated medical, psychological, and social risk factors in children with complex CHD. At 10-years of age, 125 children with CHD were assessed for general behavioral difficulties, attention deficit hyperactivity disorder (ADHD)-related behavior, and social interaction problems and were compared to normative data. Medical and cardiac factors, IQ, maternal mental health at 4 years of age and parental socioeconomic status were tested as predictors for all behavioral outcomes. Children with CHD showed no significant differences in general behavioral difficulties. However, increased ADHD-related symptoms (p &lt; 0.05) and difficulties in social interaction (p &lt; 0.05) were observed. In 23% of the children, a combination of ADHD-related symptoms and social interaction problems was reported by parents. In multivariate analyses, IQ (p &lt; 0.01) and maternal mental health (p &lt; 0.03) at 4 years of age were found to be predictive for all behavioral outcomes at 10 years while medical and cardiac risk factors were not. Our findings reveal significant difficulties in ADHD-related symptoms and social interaction problems with a significant comorbidity. Behavioral difficulties were not detected with a screening tool but with disorder-specific questionnaires. Furthermore, we demonstrate the importance of maternal mental health during early childhood on later behavioral outcomes of children with CHD. This underlines the importance of identifying and supporting parents with mental health issues at an early stage in order to support the family and improve the child's neurodevelopment.

Adults with congenital heart disease are at risk for persisting executive function deficits, which are known to affect academic achievement and quality of life. Alterations in white -matter microstructure are associated with cognitive impairments in adolescents with congenital heart disease. This study aimed to identify microstructural alterations potentially associated with executive function deficits in adults with congenital heart disease. Diffusion tensor imaging and tract-based spatial statistics were conducted in 45 patients (18 females) and 54 healthy controls (26 females) aged 18–32 years. Fractional anisotropy of white matter diffusion was compared between groups and correlated with an executive function score, derived from an extensive neuropsychological test battery. Patients showed widespread bilateral reduction in fractional anisotropy (P &lt; 0.05, multiple comparison corrected) compared to controls. Lower fractional anisotropy was driven by patients with moderate and severe defect complexity (compared to controls: P &lt; 0.001). Executive function scores were lower in patients (P &lt; 0.05) and associated with lower fractional anisotropy in the left superior corona radiata and the corticospinal tract (corrected P &lt; 0.05). Our findings confirm alterations of white matter microstructure in adults with congenital heart disease, mainly in those patients of moderate to severe complexity. These alterations are associated with impairments in executive functioning. A better understanding of the neurocognitive deficits may help counselling and care of patients with congenital heart disease across their lifespan and have the potential to improve their outcome and quality of life.