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The Boston Circulatory Arrest Study: Antecedents and Correlates of Well-Being in Adults With Congenital Heart Disease

Completed
Conditions
Transposition Great Arteries
Executive Dysfunction
Interventions
Diagnostic Test: Brain MRI, neurocognitive and psychological testing
Registration Number
NCT03073122
Lead Sponsor
Boston Children's Hospital
Brief Summary

In the landmark Boston Circulatory Arrest Study, neurologic and developmental status was measured following infant heart surgery and then prospectively at ages 1, 2.5, 4, 8, and 16 years, with findings of significant neurocognitive deficits and brain MRI abnormalities regardless of operative management. To date, no study has evaluated the neuropsychological and neuroimaging antecedents and correlates of well-being in adults with congenital heart disease, a population now \>1 million and projected to grow at 5% per year. The investigators propose to study the Boston cohort at ages 24-29 years to assess the associations of adult well-being with childhood and adolescent executive function, other measures of mental health and cognitive function, adolescent brain MRI findings, and clinical variables; findings will guide the design of interventions in childhood to optimize outcomes in adults with congenital heart disease.

Detailed Description

Previously lethal, critical congenital heart disease (CHD) can now be treated effectively with surgical, catheter, and medical interventions. The resulting dramatic improvement in life expectancy has brought a major demographic shift, so that adult patients with CHD now outnumber children with CHD, even for complex conditions. Adult survivors are at increased risk of anxiety, depression, social difficulties, lower educational attainment, and underemployment. These psychosocial morbidities may be associated with deficits in executive functions (EFs) and other neurocognitive abilities that are prevalent in children and adolescents with CHD. Deficits in EFs, represented by measures of inhibitory control, working memory, cognitive flexibility, and decision-making, are highly dependent on the integrity of cortical and subcortical neural networks that continue to develop into early adulthood and can have a major adverse impact on self-regulation and management. The goal of this proposal is to bridge the gap in knowledge between known executive function deficits in childhood CHD and adult well-being. The investigators propose to accomplish our goal by studying subjects, now age 24-29 years, who were enrolled as infants in the Boston Circulatory Arrest Study and then studied with respect to neuropsychological and developmental function at ages 1, 2.5, 4, 8, and 16 years, as well as with brain MRI at 16 years. In Aim 1, the relationship of EFs to major dimensions of adult well-being will be explored. It is hypothesized that lower performance on EFs will be related to poorer overall well-being. The outcomes measured to determine well-being will be quality of life, neuropsychological function (e.g. social cognition, memory skills), mental health diagnosis and function (e.g. anxiety, depression), social relatedness, academic achievement, and adult independence (e.g. employment status, medical follow up). In Aim 2, the relationship of EFs to MRI-derived measures of brain structure, function, and connectivity will be determined. It is hypothesized that lower performance on EFs will be associated with lower global efficiency (integration) and higher modularity (segregation). The brain MRI outcomes will be measured using global white matter connectivity, regional cortical gray matter thickness, gray matter connectivity measured from interregional correlation in cortical thickness, and functional connectivity as defined by resting state functional magnetic resonance imaging. In Aim 3, longitudinal models will be used to analyze the association of adult well-being with earlier measures of EFs and other neurocognitive and mental health variables, as well as with earlier clinical variables and adolescent neuroimaging. The associations of adult well-being dimensions with childhood and adolescent EFs, other measures of mental health and cognitive function, adolescent brain MRI findings, and clinical variables will be explored. The ultimate goal is to identify early, modifiable risk factors for adult performance to guide the design of targeted treatment strategies that optimize educational achievement, employability, and quality of life in the burgeoning population of adults with CHD.

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
127
Inclusion Criteria

Not provided

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Exclusion Criteria

Not provided

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Study & Design

Study Type
OBSERVATIONAL
Study Design
Not specified
Arm && Interventions
GroupInterventionDescription
TGA CaseBrain MRI, neurocognitive and psychological testingBrain MRI, Neurocognitive and psychological testing
ControlBrain MRI, neurocognitive and psychological testingBrain MRI, Neurocognitive and psychological testing
Primary Outcome Measures
NameTimeMethod
Quality of Life health relatedbaseline

Linear analog scale, Quality of Life Survey

Secondary Outcome Measures
NameTimeMethod
Mental health - Anxietybaseline

Hospital Anxiety and Depression scale and the structured clinical interview for DSM-V

Mental Health - Depressionbaseline

Hospital Anxiety and Depression scale and the structured clinical interview for DSM-V

Mental health - ADHDbaseline

structured clinical interview for DSM-V and Connors' Adult ADHD Rating Scales - Screening Version

Mental health - PTSDbaseline

structured clinical interview for DSM-V

Neurocognitive - memorybaseline

1. The NIH Toolbox Picture Sequence Memory Test evaluates episodic memory.

2. The Wechsler Memory Scale IV (WMS-IV) provides a comprehensive evaluation of memory abilities in adults, including auditory, visual, immediate, and delayed memory

mental health - social relatednessbaseline

The NIH Toolbox Emotion Domain - The Social Relationships subdomain measures will be administered.

Mental health - Stressbaseline

1. The Stress \& Self-Efficacy (Perceived Stress; Self-Efficacy) subdomain of the NIH Toolbox Emotion Domain

Neurocognitive - Executive functionbaseline

1. The Delis-Kaplan Executive Function System (D-KEFS) includes nine subtests: Trail-Making, Verbal Fluency, Design Fluency, Color-Word Interference, Card Sorting, Twenty Questions, Word Context, Tower, and Proverb.

2. The NIH Toolbox Assessment of Neurological and Behavioral Function, executive function battery is a set of computerized assessments that measure: (a) Inhibitory control (Flanker Inhibitory Control and Attention Test); (b) Working Memory (List Sorting Working Memory Test); (c) Cognitive Flexibility (Dimensional Change Card Sort Test).

3. The NIH Toolbox Processing Speed assesses the time it takes to process a set amount of information. It yields a main endpoint score.

4. The Behavior Rating Inventory of Executive Function for Adults (BRIEF-A) includes 75 items that yield 9 clinical scales grouped into two indexes: Behavioral Regulation and Metacognition.

Neurocognitive - social cognitionbaseline

1. The "Reading the Mind in the Eyes" Task (revised) is a computer-administered task assessing mental state attribution.

2. The Advanced Clinical Solutions for WAIS-IV and WMS IV (ACS) is a set of tasks that assess aspects of social cognition, such as affect labeling, affect recognition from faces and prosody, identification of sarcasm, and the ability to verbalize intent of a speaker.

3. The Autism Spectrum Quotient is a self-report questionnaire covering five domains of autism spectrum disorder: social skills, communication skills, imagination, attention to detail, and attention switching/tolerance of change

Neurocognitive - Risk taking behaviorbaseline

Iowa Gambling Task (IGT)

Employment statusbaseline

Categories from the National health interview survey and Hollingshead four factor index

Trial Locations

Locations (1)

Boston Children's Hospital

🇺🇸

Boston, Massachusetts, United States

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