Neurocognitive function and health-related quality of life in adolescents and young adults with CHD with pulmonary valve dysfunction.

BACKGROUND: Neurocognitive impairment and quality of life are two important long-term challenges for patients with complex CHD. The impact of re-interventions during adolescence and young adulthood on neurocognition and quality of life is not well understood. METHODS: In this prospective longitudinal multi-institutional study, patients 13-30 years old with severe CHD referred for surgical or transcatheter pulmonary valve replacement were enrolled. Clinical characteristics were collected, and executive function and quality of life were assessed prior to the planned pulmonary re-intervention. These results were compared to normative data and were compared between treatment strategies. RESULTS: Among 68 patients enrolled from 2016 to 2020, a nearly equal proportion were referred for surgical and transcatheter pulmonary valve replacement (53% versus 47%). Tetralogy of Fallot was the most common diagnosis (59%) and pulmonary re-intervention indications included stenosis (25%), insufficiency (40%), and mixed disease (35%). There were no substantial differences between patients referred for surgical and transcatheter therapy. Executive functioning deficits were evident in 19-31% of patients and quality of life was universally lower compared to normative sample data. However, measures of executive function and quality of life did not differ between the surgical and transcatheter patients. CONCLUSION: In this patient group, impairments in neurocognitive function and quality of life are common and can be significant. Given similar baseline characteristics, comparing changes in neurocognitive outcomes and quality of life after surgical versus transcatheter pulmonary valve replacement will offer unique insights into how treatment approaches impact these important long-term patient outcomes.

Autism spectrum disorder and congenital heart disease: a narrative review of the literature.

Individuals born with congenital heart disease (CHD) are at an increased risk of developing neurodevelopmental disorders. Despite this, studies are limited in their investigation of autism spectrum disorder in the context of CHD. This review provides an overview of the literature examining autism spectrum disorder in CHD and discusses strengths, limitations, and future directions. Recent efforts have been made to extrapolate the association between CHD and symptoms of autism. Findings suggest that the core features of autism spectrum disorder are also implicated in children with CHD, namely social-cognitive weaknesses, pragmatic language differences, and social problems. Compared to norm-referenced samples, separate studies have identified divergent and overlapping neuropsychological profiles among both patient groups, yet there are no studies directly comparing the two groups. There is emerging evidence of prevalence rates of autism diagnosis in CHD showing an increased odds of having autism spectrum disorder among children with CHD relative to the general population or matched controls. There also appears to be genetic links to this overlap, with several genes identified as being tied to both CHD and autism. Together, research points to potentially shared underlying mechanisms contributing to the pathophysiology of neurodevelopmental, neuropsychological, and clinical traits in CHD and autism spectrum disorder. Future investigation delineating profiles across these patient populations can fill a significant gap in the literature and aid in treatment approaches to improve clinical outcomes.

Neuropsychological assessment in pediatric oncology survivorship care: utilization of services, results of evaluation, and educational and behavioral health outcomes.

PURPOSE: Neuropsychological evaluation has become a standard component of long-term follow-up care for survivors of pediatric cancer. The purpose of the present study was to examine access to, and benefits of, neuropsychological evaluation for survivors. METHODS: A retrospective chart review was conducted on cancer survivors who were referred for neuropsychological evaluation from a multidisciplinary long-term follow-up (LTFU) clinic approximately 5 years following treatment cessation. Descriptive statistics were calculated, and t-tests and chi-square analyses were utilized to examine variables that may impact survivors' access to neuropsychological services. RESULTS: One hundred seven survivors between 6 and 26 years old were referred for a neuropsychological evaluation. Referred male patients were less likely than female patients to schedule an evaluation. Consultation with a neuropsychologist in the LTFU clinic was related to more referrals but did not improve attrition rates (55%). Twenty-four percent of evaluated patients displayed severe cognitive impairment and 75% were diagnosed with a psychological disorder. Utilization of educational and behavioral health services did not significantly change following evaluation. CONCLUSION: Survivors' utilization of neuropsychological services is lower for males than females. The presence of a neuropsychologist in a multidisciplinary team clinic can improve identification of survivors that may benefit from neuropsychological evaluation. Many survivors did not receive recommended services, suggesting patients and families may need additional supports following evaluation. Future research should focus on improving survivors' access to neuropsychological services and identifying barriers to receiving recommended services.

Sleep and neurobehavioral functioning in survivors of pediatric cancer.

OBJECTIVES: Sleep problems are a common late effect in survivors of pediatric cancer. Growing literature suggests deficits in sleep functioning may be related to more impairing neurobehavioral outcomes. The purpose of the present study was to evaluate the prevalence of parent reported sleep concerns in survivors of pediatric cancer, as well as evaluate the relationship between sleep and neurobehavioral functioning utilizing both parent and teacher input. METHODS: The study included parent-teacher dyads of 75 survivors of pediatric cancer between the ages of six and 17 who completed a clinical neuropsychological evaluation with embedded measures of neurobehavioral functioning and sleep, including excessive daytime sleepiness and snoring. Bivariate correlations and multiple linear regressions were conducted to evaluate the relationship between sleep and neurobehavioral functioning. RESULTS: No significant difference in parent reported sleep concerns was found with regard to demographic, diagnostic, or treatment variables. Daytime sleepiness was significantly elevated for 28% of the sample; snoring was not identified as a significant concern. Daytime sleepiness was significantly associated with worse neurobehavioral outcomes as reported by parents and teachers, including inattention, hyperactivity/impulsivity, peer difficulties, anxiety, depression, somatic complaints, and aggressive behaviors. Daytime sleepiness was not significantly related to report of learning problems. CONCLUSIONS: Results further support that daytime sleepiness, as reported by parents, impacts approximately one third of survivors of pediatric cancer and is associated with worse neurobehavioral outcomes across home and school environments. As a result, it is vital that sleep functioning is a target of assessment in annual survivorship care. Future longitudinal studies are needed to further delineate the directionality of the sleep-neurobehavioral relationship in survivors.

Associations Between ADHD Subtype Symptomatology and Social Functioning in Children With ADHD, Autism Spectrum Disorder, and Comorbid Diagnosis: Utility of Diagnostic Tools in Treatment Considerations.

Objective: To assess associations between objective-/caregiver-report measures of attention functioning and social impairment among children with ADHD, autism spectrum disorder (ASD), and co-occurring ASD + ADHD. Method: Patients with ADHD (N = 27), ASD (N = 23), and ASD + ADHD (N = 44) completed measures of intellectual functioning (Wechsler tests) and attention functioning (Continuous Performance Test-Second Edition [CPT-II]) as part of a neurocognitive assessment. Caregivers completed the Conners Third Edition to assess day-to-day inattentiveness, hyperactivity/impulsivity, and the Social Responsiveness Scale (SRS) to assess social functioning. Results: Among patients with ADHD, attention measures contributed to 48% of the variance in total SRS scores, with caregiver-reported hyperactivity/impulsivity as the strongest factor. In contrast, among those with ASD + ADHD, attention measures accounted for 40% of the variance, largely due to inattention problems. No associations between domains were observed among patients with ASD. Conclusion: Differential ADHD symptoms are associated with social impairment among children with ADHD versus ASD + ADHD; whereas, no associations were observed among those with ASD.

Brief Report: Neuropsychological Testing and Informant-Ratings of Children with Autism Spectrum Disorder, Attention-Deficit/Hyperactivity Disorder, or Comorbid Diagnosis.

This study aimed to examine the neuropsychological correlates of child patients diagnosed with ADHD, autism spectrum disorder (ASD), or comorbid ASD + ADHD through a multidisciplinary ASD evaluation clinic. Patients completed standardized tests of intellectual, attention, social-affective/cognitive, and executive functioning; and a semi-structured assessment commonly used for autism diagnosis. The majority of patients were medicated for ADHD concerns during testing. Parents and teachers also completed inventories of day-to-day social and attentional functioning. Group effects were found across objective social measures but not across related respondent-ratings. In contrast, group differences were observed in parent-ratings of attention difficulties, but not on standardized tests of attention or executive functioning. Findings underscore importance of integrating objective and functional measures when assessing ASD and/or ADHD.

Visual Attention and Math Performance in Survivors of Childhood Acute Lymphoblastic Leukemia.

OBJECTIVE: Attentional and academic difficulties, particularly in math, are common in survivors of childhood acute lymphoblastic leukemia (ALL). Of cognitive deficits experienced by survivors of childhood ALL, attention deficits may be particularly responsive to intervention. However, it is unknown whether deficits in particular aspects of attention are associated with deficits in math skills. The current study investigated relationships between math calculation skills, performance on an objective measure of sustained attention, and parent- and teacher-reported attention difficulties. METHOD: Twenty-four survivors of childhood ALL (Mage = 13.5 years, SD = 2.8 years) completed a computerized measure of sustained attention and response control and a written measure of math calculation skills in the context of a comprehensive clinical neuropsychological evaluation. Parent and teacher ratings of inattention and impulsivity were obtained. RESULTS: Visual response control and visual attention accounted for 26.4% of the variance observed among math performance scores after controlling for IQ (p < .05). Teacher-rated, but not parent-rated, inattention was significantly negatively correlated with math calculation scores. CONCLUSIONS: Consistency of responses to visual stimuli on a computerized measure of attention is a unique predictor of variance in math performance among survivors of childhood ALL. Objective testing of visual response control, rather than parent-rated attentional problems, may have clinical utility in identifying ALL survivors at risk for math difficulties.

[Formula: see text]Congenital heart disease: A primer for the pediatric neuropsychologist.

Congenital heart disease (CHD) affects millions of people worldwide, including over one million children in the United States. Approximately 25% of children born with CHD require intensive surgical intervention within the first year of life. Despite improved rates of survival into adulthood - rates that exceed 90% in the modern era - children and adolescents with CHD remain at risk for neurological injury and a range of neurobehavioral and psychosocial challenges that pose a threat to quality of life across the lifespan. Consequently, as experts in both clinical psychology and brain development, neuropsychologists are becoming increasingly involved in cardiac follow-up and monitoring to promote optimal developmental outcomes. The primary objective of this paper is to provide an evidence-based, clinically-oriented primer on CHD for pediatric neuropsychologists working with this growing population of survivors. Following an introduction to current standard-of-care guidelines for managing children and adolescents with CHD, we present an overview of brain development within the context of CHD, review neuropsychological outcomes, examine factors influencing variability in outcomes, and discuss implications and strategies for clinical assessment.