Congenital Heart Disease: Impact on Learning and Development in Down Syndrome (CHILD-DS)

NCT ID: NCT05312177

Last Updated: 2024-01-11

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

COMPLETED

Total Enrollment

273 participants

Study Classification

OBSERVATIONAL

Study Start Date

2022-05-01

Study Completion Date

2023-12-31

Brief Summary

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The study objective is to compare neurodevelopmental (ND) and behavioral outcomes between children with Down syndrome (DS) who had complete atrioventricular septal defect (CAVSD) repair and children from the same clinical sites with DS without major congenital heart disease (CHD) requiring previous or planned CHD surgery.

Detailed Description

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We are conducting a multicenter cohort study, ancillary to the Pediatric Heart Network (PHN) Residual Lesion Score (RLS) study, to investigate determinants of ND and behavioral outcomes in children with DS, focusing on the role of CHD surgery. We are including children with DS who had CAVSD repair, as this group comprises 91% of all children with DS in the RLS Study, with similar underlying congenital cardiac defect and surgical repair complexity, allowing our children with DS and CHD to be a homogeneous group. Moreover, one in five individuals with DS is born with an AVSD, a 2000 times higher incidence than in those with normal chromosomes. In addition to recruiting children with DS who had CAVSD repair, we will recruit similarly-aged children with DS who do not have documented major CHD (i.e., CHD requiring previous or planned CHD surgery) to come into the same PHN site for a single study visit consisting of detailed phenotyping by completion of a Health \& Developmental History Intake form, ND and behavioral assessments, and optional collection and storage of saliva specimens in the PHN Biorepository. By building our sample from RLS Study participants and recruiting additional children who had infant CAVSD repair and meet study eligibility criteria, as well as a comparison group from the same PHN sites, we are leveraging rich prospective data in a group of DS children with CAVSD repair from a nationally representative sample, and the expertise of the PHN and Boston Children's Hospital (BCH).

Conditions

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Down Syndrome Congenital Heart Disease

Study Design

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Observational Model Type

CASE_CONTROL

Study Time Perspective

PROSPECTIVE

Study Groups

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DS with CAVSD Repair

Children ages 5 through 12 years with Down syndrome who had Complete atrioventricular septal defect (CAVSD) repair in the first year of life and their parent(s) will be administered the Stanford-Binet Intelligence Scales, Fifth Edition (SB-5), Peabody Picture Vocabulary Test, Fifth Edition (PPVT-5), Expressive Vocabulary Test, Third Edition (EVT-3), Leiter International Performance Scale, Third Edition (Leiter-3), Vineland-3 Q-global Comprehensive Report (Vineland-3) ,Social Communication Questionnaire, Current version (SCQ), Aberrant Behavior Checklist, Second Edition, (ABC-2) and Repetitive Behavior Scale, Revised (RBS-R)

Stanford Binet Intelligence Scales, Fifth Edition

Intervention Type OTHER

The SB-5 is a comprehensive measure of cognitive and intellectual functioning for individuals from age two through 85 years of age. The SB-5 individually assess a child's functioning in five areas of cognitive functioning: Fluid Reasoning, Knowledge, Quantitative Reasoning, Visual Spatial, and Working Memory. The SB-5 is well established in the existing research literature as an assessment tool to evaluate developmental and intellectual abilities in children with ND disorders.

Vineland Adaptive Behavior Scales Third Edition

Intervention Type OTHER

The VABS-3 are a parent report, interview style measure of adaptive behavior for individuals ages birth through 90 months. The VABS-3 assesses adaptive behavior in four broad domains of Communication, Daily Living Skills, Socialization, and Motor Skills, and includes a Maladaptive Behavior Domain that assesses problem behaviors.

Peabody Picture Vocabulary Test, Fifth Edition

Intervention Type OTHER

The PPVT-5 is a norm-referenced language measure that evaluates single-word receptive vocabulary. The PPVT-5 provides standard scores for individuals ages 2.6 through 90 years.

Expressive Vocabulary Test, Third Edition

Intervention Type OTHER

The EVT-3 is a norm-referenced language measure that evaluates single-word expressive vocabulary. The EVT-3 provides standard scores for individuals ages 2.6 through 90 years.

Leiter International Performance Scale, Third Edition

Intervention Type OTHER

The Leiter-3 is a nonverbal measure of cognitive functioning and fluid reasoning skills in individuals ages 3 through 75 years. The Leiter-3 measures nonverbal IQ across four subscales, including Sequential Order (SO), Form Completion (FC), Classification and Analogies (CA), and Figure Ground (FG).

Social Communication Questionnaire, Current Form

Intervention Type OTHER

The SCQ is a parent-report measure of social communication, social interactions, play, and behavior.

Aberrant Behavior Checklist

Intervention Type OTHER

The ABC-2 is a parent report measure of problematic behavior at home and in the community. It measures behavior on five subscales (1) Irritability, (2) Social Withdrawal, (3) Stereotypic Behavior, (4) Hyperactivity/Noncompliance, and (5) Inappropriate Speech.

Repetitive Behavior Scale, Revised

Intervention Type OTHER

The RBS-R is a parent report measure that comprehensively surveys for the presence of repetitive behaviors.

DS without major CHD

Children ages 5 through 12 years with Down syndrome without major Congenital Heart Disease(CHD) and their parent(s) will be administered the Stanford-Binet Intelligence Scales, Fifth Edition (SB-5), Peabody Picture Vocabulary Test, Fifth Edition (PPVT-5), Expressive Vocabulary Test, Third Edition (EVT-3), Leiter International Performance Scale, Third Edition (Leiter-3), Vineland-3 Q-global Comprehensive Report (Vineland-3) ,Social Communication Questionnaire, Current version (SCQ), Aberrant Behavior Checklist, Second Edition, (ABC-2) and Repetitive Behavior Scale, Revised (RBS-R)

Stanford Binet Intelligence Scales, Fifth Edition

Intervention Type OTHER

The SB-5 is a comprehensive measure of cognitive and intellectual functioning for individuals from age two through 85 years of age. The SB-5 individually assess a child's functioning in five areas of cognitive functioning: Fluid Reasoning, Knowledge, Quantitative Reasoning, Visual Spatial, and Working Memory. The SB-5 is well established in the existing research literature as an assessment tool to evaluate developmental and intellectual abilities in children with ND disorders.

Vineland Adaptive Behavior Scales Third Edition

Intervention Type OTHER

The VABS-3 are a parent report, interview style measure of adaptive behavior for individuals ages birth through 90 months. The VABS-3 assesses adaptive behavior in four broad domains of Communication, Daily Living Skills, Socialization, and Motor Skills, and includes a Maladaptive Behavior Domain that assesses problem behaviors.

Peabody Picture Vocabulary Test, Fifth Edition

Intervention Type OTHER

The PPVT-5 is a norm-referenced language measure that evaluates single-word receptive vocabulary. The PPVT-5 provides standard scores for individuals ages 2.6 through 90 years.

Expressive Vocabulary Test, Third Edition

Intervention Type OTHER

The EVT-3 is a norm-referenced language measure that evaluates single-word expressive vocabulary. The EVT-3 provides standard scores for individuals ages 2.6 through 90 years.

Leiter International Performance Scale, Third Edition

Intervention Type OTHER

The Leiter-3 is a nonverbal measure of cognitive functioning and fluid reasoning skills in individuals ages 3 through 75 years. The Leiter-3 measures nonverbal IQ across four subscales, including Sequential Order (SO), Form Completion (FC), Classification and Analogies (CA), and Figure Ground (FG).

Social Communication Questionnaire, Current Form

Intervention Type OTHER

The SCQ is a parent-report measure of social communication, social interactions, play, and behavior.

Aberrant Behavior Checklist

Intervention Type OTHER

The ABC-2 is a parent report measure of problematic behavior at home and in the community. It measures behavior on five subscales (1) Irritability, (2) Social Withdrawal, (3) Stereotypic Behavior, (4) Hyperactivity/Noncompliance, and (5) Inappropriate Speech.

Repetitive Behavior Scale, Revised

Intervention Type OTHER

The RBS-R is a parent report measure that comprehensively surveys for the presence of repetitive behaviors.

Interventions

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Stanford Binet Intelligence Scales, Fifth Edition

The SB-5 is a comprehensive measure of cognitive and intellectual functioning for individuals from age two through 85 years of age. The SB-5 individually assess a child's functioning in five areas of cognitive functioning: Fluid Reasoning, Knowledge, Quantitative Reasoning, Visual Spatial, and Working Memory. The SB-5 is well established in the existing research literature as an assessment tool to evaluate developmental and intellectual abilities in children with ND disorders.

Intervention Type OTHER

Vineland Adaptive Behavior Scales Third Edition

The VABS-3 are a parent report, interview style measure of adaptive behavior for individuals ages birth through 90 months. The VABS-3 assesses adaptive behavior in four broad domains of Communication, Daily Living Skills, Socialization, and Motor Skills, and includes a Maladaptive Behavior Domain that assesses problem behaviors.

Intervention Type OTHER

Peabody Picture Vocabulary Test, Fifth Edition

The PPVT-5 is a norm-referenced language measure that evaluates single-word receptive vocabulary. The PPVT-5 provides standard scores for individuals ages 2.6 through 90 years.

Intervention Type OTHER

Expressive Vocabulary Test, Third Edition

The EVT-3 is a norm-referenced language measure that evaluates single-word expressive vocabulary. The EVT-3 provides standard scores for individuals ages 2.6 through 90 years.

Intervention Type OTHER

Leiter International Performance Scale, Third Edition

The Leiter-3 is a nonverbal measure of cognitive functioning and fluid reasoning skills in individuals ages 3 through 75 years. The Leiter-3 measures nonverbal IQ across four subscales, including Sequential Order (SO), Form Completion (FC), Classification and Analogies (CA), and Figure Ground (FG).

Intervention Type OTHER

Social Communication Questionnaire, Current Form

The SCQ is a parent-report measure of social communication, social interactions, play, and behavior.

Intervention Type OTHER

Aberrant Behavior Checklist

The ABC-2 is a parent report measure of problematic behavior at home and in the community. It measures behavior on five subscales (1) Irritability, (2) Social Withdrawal, (3) Stereotypic Behavior, (4) Hyperactivity/Noncompliance, and (5) Inappropriate Speech.

Intervention Type OTHER

Repetitive Behavior Scale, Revised

The RBS-R is a parent report measure that comprehensively surveys for the presence of repetitive behaviors.

Intervention Type OTHER

Other Intervention Names

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SB-5 VABS-3 PPVT-5 EVT-3 Leiter-3 SCQ ABC-2 RBS-R

Eligibility Criteria

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Inclusion Criteria

Down Syndrome CAVSD Repair Group:

* Trisomy 21
* Male or Female, ages 5 years through 12 years
* Had CAVSD repair within the first year of life
* Parent or guardian and patient willing to comply with protocol and complete all study assessments; parent or guardian willing to provide written informed consent
* Child able to speak and understand English

Down Syndrome Comparison Group

* Trisomy 21
* Male or Female, age 5 years through 12 years
* No major CHD, defined as CHD requiring previous or planned CHD surgery
* Parent or guardian and patient willing to comply with protocol and complete all study assessments; parent or guardian willing to provide written informed consent
* Child able to speak and understand English

Exclusion Criteria

Both Groups

\- Mosaic DS

Down Syndrome CAVSD Repair Group only - Did not have CAVSD repair in the first year of life

Down Syndrome Comparison Group only

\- Major CHD requiring previous or planned CHD surgery - i.e., CHD surgery occurring (a) in the period between birth and time of recruitment into the CHILD-DS Study, or (b) planned for a future date.
Minimum Eligible Age

60 Months

Maximum Eligible Age

155 Months

Eligible Sex

ALL

Accepts Healthy Volunteers

Yes

Sponsors

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National Heart, Lung, and Blood Institute (NHLBI)

NIH

Sponsor Role collaborator

National Institutes of Health (NIH)

NIH

Sponsor Role collaborator

Carelon Research

OTHER

Sponsor Role lead

Responsible Party

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Responsibility Role SPONSOR

Principal Investigators

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Maria VanRompay, PhD

Role: PRINCIPAL_INVESTIGATOR

Carelon Research

Locations

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Alfred I. duPont Hospital for Children

Wilmington, Delaware, United States

Site Status

Children's National Hospital

Washington D.C., District of Columbia, United States

Site Status

Children's Healthcare of Atlanta at Egleston

Atlanta, Georgia, United States

Site Status

Riley Children's Hospital

Indianapolis, Indiana, United States

Site Status

Boston Children's Hospital

Boston, Massachusetts, United States

Site Status

University of Michigan Health System/Mott Hospital

Ann Arbor, Michigan, United States

Site Status

Children's Mercy Hospital

Kansas City, Missouri, United States

Site Status

Columbia College of Physicians and Surgeons

New York, New York, United States

Site Status

Cincinnati Children's Hospital Medical Center

Cincinnati, Ohio, United States

Site Status

Children's Hospital of Philadelphia

Philadelphia, Pennsylvania, United States

Site Status

Medical University of South Carolina

Charleston, South Carolina, United States

Site Status

Baylor College of Medicine - Texas Children's Hospital

Houston, Texas, United States

Site Status

Primary Children's Hospital

Salt Lake City, Utah, United States

Site Status

Hospital for Sick Children

Toronto, Ontario, Canada

Site Status

Countries

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United States Canada

Other Identifiers

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3U24HL135691-03S1

Identifier Type: NIH

Identifier Source: secondary_id

View Link

CHILD-DS

Identifier Type: -

Identifier Source: org_study_id

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