Johanna Calderon

Executive function and theory of mind in school-aged children after neonatal corrective cardiac surgery for transposition of the great arteries

Aim  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.

Impact of prenatal diagnosis on neurocognitive outcomes in children with transposition of the great arteries.

OBJECTIVES To assess the effect of prenatal diagnosis of congenital heart disease on neurocognitive outcomes in children with d-transposition of the great arteries (TGA) after surgical correction. STUDY DESIGN A prospective study of children born with a TGA between 2003 and 2005 and aged 4 to 6 years was conducted. General intelligence, language, executive functions, and social cognition scores and preoperative, intraoperative, and postoperative factors were evaluated according to time of TGA diagnosis. Neurocognitive data were also compared with a control group. RESULTS Forty-five eligible patients (67% male) were examined; 29 had a prenatal diagnosis of TGA and 16 did not. All children were comparable in age, sex, and demographic variables. Diagnostic groups did not differ in preoperative, intraoperative, and postoperative variables. Preoperative acidosis was more frequent in the postnatal group (18% versus 3%). All patients had normal IQ scores, language, and verbal working memory. However, neurocognitive deficits were more prevalent and more severe in children with a postnatal-TGA. Prenatal diagnosis was associated with better outcomes in executive functions. CONCLUSIONS Prenatal diagnosis of TGA is associated with better neurocognitive outcomes. Time of diagnosis may influence the development of early complex cognitive skills such as executive functions.

Executive function deficits in congenital heart disease: why is intervention important?

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 childrens 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.

Executive Functions Development in 5- to 7-Year-Old Children With Transposition of the Great Arteries: A Longitudinal Study

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.

Psychiatric Disorders in Adolescents With Single Ventricle Congenital Heart Disease

Examination of adolescents with critical congenital heart disease revealed high risk for certain psychiatric morbidities, providing insight into clinical assessment and management of these youth. 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 < .001). Specifically, they had higher rates of lifetime anxiety disorder and ADHD (P < .001 each). The CHD group scored lower on the primary psychosocial functioning measure, the Children’s Global Assessment Scale, than referents (CHD median [interquartile range]: 62 [54–66], referent: 85 [73–90]; P < .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 psychosocial functioning 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 high risk of psychiatric morbidity, particularly anxiety disorders and ADHD. Early identification of psychiatric symptoms is critical to the management of patients with CHD.

Use of Early Remedial Services in Children with Transposition of the Great Arteries

OBJECTIVES To characterize the prevalence of use of early remedial services and its associated demographic, medical, and cognitive factors in children aged 4-6 years with corrected transposition of the great arteries (TGA). STUDY DESIGN This was a prospective study of neurocognitive outcomes after TGA. Children underwent formal neuropsychological testing including general intelligence and a comprehensive battery of executive functions (EF) including motor and interference control, short-term memory, and working memory as well as cognitive flexibility. Parental reports on the childrens behavior and EF were also evaluated. Demographic factors and preoperative, intraoperative, and postoperative factors as well as cognitive factors were examined according to the current use of remediation. RESULTS Forty-five patients (67% male) and their parents participated in this study. Twenty-four (53%) patients were receiving remedial services. Male sex, a postnatal diagnosis of TGA, and a longer postoperative intensive care unit stay were significantly associated with use of remediation. Children receiving remediation had lower EF scores, had more severe EF deficits as observed by formal testing, and were rated as having more behavioral daily life difficulties. However, in the group without remediation, 13 children (43%) also displayed EF deficits rated as moderate to severe. CONCLUSIONS Demographic and medical factors could help identify children at higher risk for neurocognitive delays. Evaluation of executive functioning from an early age may influence referral for remediation.

Facial Expression Recognition and Emotion Understanding in Children after Neonatal Open-Heart Surgery for Transposition of the Great Arteries

Theory of mind impairments are part of the cognitive morbidities associated with transposition of the great arteries (TGA). We sought to assess core components of social cognition in school‐aged children with TGA.

Cognitive outcomes and health-related quality of life in adults two decades after the arterial switch operation for transposition of the great arteries

Objective: The study objective was to evaluate the cognitive and psychosocial outcomes of young adults who underwent an arterial switch operation for transposition of the great arteries. Methods: Sixty‐seven adults with transposition of the great arteries (aged 22.9 ± 3.3 years) and 43 healthy subjects matched in age, gender, and educational level participated. Global cognitive functioning, psychologic state, and health‐related quality of life were evaluated using validated tools. We analyzed patient‐related and procedure‐related risk factors associated with cognitive and psychosocial outcomes. Results: Some 69% of patients had an intelligence quotient greater than 85 (normal range, 85‐115). Mean full‐scale, verbal, and performance intelligence quotients were lower in patients (94.9 ± 15.3, 96.8 ± 16.2, 93.7 ± 14.6, respectively) than in healthy subjects (103.4 ± 12.3, P = .003; 102.5 ± 11.5, P = .033; 103.8 ± 14.3, P < .001, respectively). Cognitive difficulties (intelligence quotient ≤−1 standard deviation) and impairments (intelligence quotient ≤−2 standard deviations) were more frequent in patients than in the general population (31% vs 16%, P = .001; 6% vs 2%, P = .030). Patients with cognitive difficulties had lower educational level (P < .001) and more grade retention at school (P = .007). Patients reported an overall satisfactory health‐related quality of life; however, those with cognitive or psychologic difficulties reported poorer quality of life. Predictors of worse outcomes included lower parental socioeconomic and educational status, older age at surgery, and longer hospitalization stay. Conclusions: Despite satisfactory outcomes in most adults with transposition of the great arteries, a substantial proportion has cognitive or psychologic difficulties that may reduce their academic success and quality of life. Further studies are needed to better understand the long‐term outcome of this population to provide prevention, surveillance, and care strategies.

Ages and Stages Questionnaire at 3 Years for Predicting IQ at 5–6 Years

This study assesses the predictive value of the 36-month Ages & Stages Questionnaires score for IQ at age 5 to 6 years in a sample of the general population. OBJECTIVES: To assess the predictive value of the 36-month Ages & Stages Questionnaire (ASQ) score for IQ score at age 5 to 6 years in the general population and to identify factors associated with IQ <85 once the ASQ score is taken into account. METHODS: Data were collected from 939 children enrolled in a population-based prospective cohort study. Developmental outcomes at 36 months were assessed via the ASQ and at 5 to 6 years via the Wechsler Preschool and Primary Scale of Intelligence. The ASQ threshold was identified via the receiver operating characteristic curve. Additional predictive factors to obtain an IQ <85 were investigated, and their interaction with ASQ score was studied. RESULTS: Sixty-nine children (7.3%) had an IQ <85. A 36-month ASQ score threshold of 270 was optimal to identify children with an IQ <85 at 5 to 6 years, with a 0.77 ± 0.11 sensitivity and 0.68 ± 0.03 specificity. Maternal educational level and occupational activity at the time of ASQ completion were associated with the risk of an IQ <85 at a given ASQ level. In the multivariate model, no interaction between the studied factors and ASQ score reached significance. CONCLUSIONS: In the general pediatric population, 36-month ASQ parental reports could be used to identify children at later risk of cognitive delay. Low maternal education level should also be considered as a major risk factor for lower IQ in preschool children regardless of ASQ score.

Population-based study of cognitive outcomes in congenital heart defects

Objective To characterise and compare cognitive outcomes in children with operated (open-heart surgery) and non-operated (catheter-based interventions only or no intervention) congenital heart defects (CHD) and to determine associated risk factors. Design This prospective population-based study reports outcomes of 3-year-old children with CHD with or without open-heart surgery. Main outcome measures Standardised cognitive scores (mean scores and proportions below normative values) were assessed with the Kaufman Assessment Battery for Children II. We analysed demographic, perinatal and operative variables as predictors of cognitive outcomes. Results 419 children participated (154 with open-heart surgery; 265 without surgery). Global cognitive scores did not differ between the groups. Compared with the non-operated group, children who underwent surgery obtained lower scores in expressive language (p=0.03) and logical reasoning (p=0.05). When compared with test norms, the frequency of global cognitive scores >1 SDs below the expected mean was higher in the surgical group (25% vs 16% in the general population) (p=0.03). A higher-than-expected proportion of children in the non-operated group scored >2 SDs below the expected mean (7% vs 2%) (p=0.05). Being small for gestational age (SGA) significantly increased the risk of cognitive impairment in the surgical group, after adjustments for multiple covariates including maternal education, complexity of the CHD and operative-related variables (adjusted OR=5.9; 95% CI (1.7 to 20.1)). Conclusions Despite mean scores within the normative range, a high proportion of preschool children with CHD with or without surgery are at early cognitive risk. SGA is a strong predictor of the neurodevelopmental prognosis in CHD.