Executive Function and Psychosocial Quality of Life in School Age Children with Congenital Heart Disease.

OBJECTIVE: To test a model to predict psychosocial quality of life (QOL) in children with congenital heart disease (CHD) via executive dysfunction. STUDY DESIGN: Parents of 91 children with CHD requiring surgery in the first year of life completed questionnaires by mail or as part of their cardiology clinic visit. Latent class analysis identified 2 groups of patients with different likelihoods of executive dysfunction. Select medical and demographic characteristics and executive dysfunction group membership were evaluated as predictors of QOL using structural equation modeling. RESULTS: In children with CHD, aortic obstruction, male sex, and premature birth predicted worse executive function, explaining 59% of the variance. Structural equation modeling results indicated that executive dysfunction plays an important mediating role, through which CHD with aortic obstruction, male sex, and premature birth indirectly affect psychosocial QOL. Neurologic abnormalities and single-ventricle CHD did not significantly predict executive dysfunction or QOL. CONCLUSIONS: Executive dysfunction is a strong predictor of psychosocial QOL at school age. Select medical and demographic risk factors did not directly predict QOL at school age in CHD; however, aortic obstruction, premature birth, and male sex impacted QOL indirectly by contributing to executive dysfunction. These findings suggest important risk factors for executive dysfunction that can be monitored, allowing for provision of early supports for executive skills development in an effort to improve long term psychosocial QOL in at-risk children with CHD.

Adding the missing voice: How self-report of autistic youth self-report on an executive functioning rating scale compares to parent report and that of youth with attention deficit hyperactivity disorder or neurotypical development.

LAY ABSTRACT: Executive functions are often impaired in autistic people and relate to important outcomes such as mental health, success in school and work, and quality of life. Evaluating executive functions helps autistic people, clinicians, and families identify targets for external supports and skill building. Youth self-report of executive function has not been studied, yet we know that self-report from autistic youth is key to understanding other cognitive/behavioral phenomena in autism such as anxiety, obsessions/compulsions, sensory sensitivities, and repetitive behaviors. We investigated self- and parent-report of executive function problems in 197 autistic youth without intellectual disability (ages 11-18 years), including the magnitude and profiles of executive function problems autistic youth report across subdomains of executive function. We compared autistic self-report with that of 114 youth with attention deficit hyperactivity disorder without intellectual disability and 197 neurotypical youth. We found that autistic youth report significant executive function problems compared to neurotypical youth and a distinctive profile of challenges in comparison to attention deficit hyperactivity disorder youth. Parents and their autistic children diverged regarding the magnitude of the youth's executive function difficulties, but both identify inflexibility as the most impaired executive function subdomain. Autistic youth and their parents were somewhat more concordant in their report of executive function problems than youth with attention deficit hyperactivity disorder and their parents, but only showed moderate concordance at best. These findings elevate the importance of asking autistic youth directly about their executive functioning when engaging them in assessment and intervention, or researching executive functions in autism.

Prevalence and pattern of executive dysfunction in school age children with congenital heart disease.

OBJECTIVE: Executive function, a set of cognitive skills important to social and academic outcomes, is a specific area of cognitive weakness in children with congenital heart disease (CHD). We evaluated the prevalence and profile of executive dysfunction in a heterogeneous sample of school aged children with CHD, examined whether children with executive dysfunction are receiving school services and support, and identified risk factors for executive dysfunction at school age. DESIGN: Ninety-one school aged patients completed questionnaires, including the Behavior Rating Inventory of Executive Function (BRIEF) and a medical history questionnaire. An age- and gender- matched control sample was drawn from a normative database. RESULTS: Children with CHD had a higher rate of parent reported executive dysfunction (OR = 4.37, P < .0001), especially for working memory (OR = 8.22, P < .0001) and flexibility (OR = 8.05, P < .0001). Those with executive dysfunction were not more likely to be receiving school services (P > .05). Gender, premature birth (≤37 weeks), and CHD with aortic obstruction were predictive of executive dysfunction, especially for behavior regulation skills. CONCLUSIONS: School aged children with CHD have an increased prevalence of executive dysfunction, especially problems with working memory and flexibility, and are underserved by the school system. The increased risk for executive dysfunction in those with CHD and prematurity or CHD with aortic obstruction suggests an etiology of delayed brain development in the fetal and neonatal periods, while male gender may increase susceptibility to brain injury. This study highlights the need for regular neurodevelopmental follow up in children with CHD, and a need to better understand mechanisms that contribute to adverse neurodevelopmental outcomes.

Sex differences in parent-reported executive functioning and adaptive behavior in children and young adults with autism spectrum disorder.

This study is the largest to date examining executive function and adaptive skills in females with autism spectrum disorder (ASD). Its primary aim was to utilize parent ratings of real-world executive functioning and adaptive behavior to better understand whether females with ASD differ from males with ASD in these areas of everyday functioning. We compared 79 females with ASD to 158 males with ASD (ages 7-18) who were statistically matched on age, IQ, and level of ADHD or ASD traits. All participants were assessed using the Behavior Rating Inventory of Executive Function (BRIEF) and a subset (56 females and 130 males) also received the Vineland Adaptive Behavior Scales (VABS). Females were rated by parents as having greater problems with executive function on the BRIEF. Parents also rated females as exhibiting more difficulties than males on the Daily Living Skills domain of the VABS. There was a correlation between increased global EF difficulty and decreased adaptive ability in both males and females. Our results indicate relative weaknesses for females compared to males diagnosed with ASD on executive function and daily living skills. These differences occur in the absence of sex differences in our sample in age, IQ, clinician ratings of core ASD symptomatology, parent ratings of ADHD symptoms, and parent-reported social and communication adaptive skills on the VABS. These findings indicate specific liabilities in real world EF and daily living skills for females with ASD and have important implications for targeting their treatments. Autism Res 2017, 10: 1653-1662. © 2017 International Society for Autism Research, Wiley Periodicals, Inc.

The Flexibility Scale: Development and Preliminary Validation of a Cognitive Flexibility Measure in Children with Autism Spectrum Disorders.

Flexibility is a key component of executive function, and is related to everyday functioning and adult outcomes. However, existing informant reports do not densely sample cognitive aspects of flexibility; the Flexibility Scale (FS) was developed to address this gap. This study investigates the validity of the FS in 221 youth with ASD and 57 typically developing children. Exploratory factor analysis indicates a five-factor scale: Routines/rituals, transitions/change, special interests, social flexibility, and generativity. The FS demonstrated convergent and divergent validity with comparative domains of function in other measures, save for the Generativity factor. The FS discriminated participants with ASD and controls. Thus, this study suggests the FS may be a viable, comprehensive measure of flexibility in everyday settings.