NCT05312177

Brief Summary

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.

Trial Health

90
On Track

Trial Health Score

Automated assessment based on enrollment pace, timeline, and geographic reach

Enrollment
273

participants targeted

Target at P75+ for all trials

Timeline
Completed

Started May 2022

Geographic Reach
2 countries

14 active sites

Status
completed

Health score is calculated from publicly available data and should be used for screening purposes only.

Trial Relationships

Click on a node to explore related trials.

Study Timeline

Key milestones and dates

First Submitted

Initial submission to the registry

February 24, 2020

Completed
2.1 years until next milestone

First Posted

Study publicly available on registry

April 5, 2022

Completed
26 days until next milestone

Study Start

First participant enrolled

May 1, 2022

Completed
1.7 years until next milestone

Primary Completion

Last participant's last visit for primary outcome

December 31, 2023

Completed
Same day until next milestone

Study Completion

Last participant's last visit for all outcomes

December 31, 2023

Completed
Last Updated

January 11, 2024

Status Verified

October 1, 2023

Enrollment Period

1.7 years

First QC Date

February 24, 2020

Last Update Submit

January 10, 2024

Conditions

Keywords

Down SyndromeCHDCongenital Heart DiseasePediatric Heart NetworkINCLUDE

Outcome Measures

Primary Outcomes (5)

  • Neurodevelopmental outcomes between children with DS who had CAVSD repair and children from the same clinical sites with DS without major CHD.

    Verbal and non-verbal ratio intelligent quotients (IQs) derived from the Stanford Binet Intelligence Scales will be compared between the children with DS and CAVSD repair and the children with DS without major CHD.

    1 day

  • Behavioral outcomes between children with DS who had CAVSD repair and children from the same clinical sites with DS without major CHD.

    Adaptive composite scores from the Vineland Adaptive Behavior Scales will be compared between the children with DS and CAVSD repair and the children with DS without major CHD.

    1 day

  • Language abilities compared between the children with DS and CAVSD repair and the children with DS without major CHD.

    Using the Peabody Picture Vocabulary Test, Expressive Vocabulary Test, and Leiter International Performance Scale total language, auditory comprehension, and expressive communication will be compared between the children with DS and CAVSD repair and the children with DS without major CHD.

    1 day

  • Emotional outcomes compared between the children with DS and CAVSD repair and the children with DS without major CHD.

    Using the Repetitive Behavior Scale and Aberrant Behavior Checklist , behavioral and emotional problems will be compared will be compared between the children with DS and CAVSD repair and the children with DS without major CHD.

    1 day

  • Social Communication compared between the children with DS and CAVSD repair and the children with DS without major CHD.

    Using the Social Communication Questionnaire, social communication, social interactions, play and behavior will be compared between the children with DS and CAVSD repair and the children with DS without major CHD.

    1 day

Secondary Outcomes (2)

  • Comorbidities as predictors of neurodevelopment and behavior

    1 day

  • Sociodemographic factors as predictors of neurodevelopment and behavior

    1 day

Study Arms (2)

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)

Other: Stanford Binet Intelligence Scales, Fifth EditionOther: Vineland Adaptive Behavior Scales Third EditionOther: Peabody Picture Vocabulary Test, Fifth EditionOther: Expressive Vocabulary Test, Third EditionOther: Leiter International Performance Scale, Third EditionOther: Social Communication Questionnaire, Current FormOther: Aberrant Behavior ChecklistOther: Repetitive Behavior Scale, Revised

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)

Other: Stanford Binet Intelligence Scales, Fifth EditionOther: Vineland Adaptive Behavior Scales Third EditionOther: Peabody Picture Vocabulary Test, Fifth EditionOther: Expressive Vocabulary Test, Third EditionOther: Leiter International Performance Scale, Third EditionOther: Social Communication Questionnaire, Current FormOther: Aberrant Behavior ChecklistOther: Repetitive Behavior Scale, Revised

Interventions

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.

Also known as: SB-5
DS with CAVSD RepairDS without major CHD

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.

Also known as: VABS-3
DS with CAVSD RepairDS without major CHD

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.

Also known as: PPVT-5
DS with CAVSD RepairDS without major CHD

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.

Also known as: EVT-3
DS with CAVSD RepairDS without major CHD

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).

Also known as: Leiter-3
DS with CAVSD RepairDS without major CHD

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

Also known as: SCQ
DS with CAVSD RepairDS without major CHD

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.

Also known as: ABC-2
DS with CAVSD RepairDS without major CHD

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

Also known as: RBS-R
DS with CAVSD RepairDS without major CHD

Eligibility Criteria

Age60 Months - 155 Months
Sexall
Healthy VolunteersYes
Age GroupsChild (0-17)
Sampling MethodNon-Probability Sample
Study Population

PHN RLS Study participants with DS and CAVSD repair from the PHN sites are eligible to participate in this study. Children with a diagnosis of mosaic DS will be excluded, as these forms of DS show different ND trajectories and are usually excluded. While data on mosaic DS were not collected in the RLS Study, we anticipate few if any to have this diagnosis. The DS comparison group will be required to have no major CHD, defined as CHD that requires CHD surgery (a) in the period between birth and recruitment into the CHILD-DS Study or (b) planned for a future date.

You may qualify if:

  • 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

You may not qualify if:

  • 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.

Contact the study team to confirm eligibility.

Sponsors & Collaborators

Study Sites (14)

Alfred I. duPont Hospital for Children

Wilmington, Delaware, 19899, United States

Location

Children's National Hospital

Washington D.C., District of Columbia, 20010, United States

Location

Children's Healthcare of Atlanta at Egleston

Atlanta, Georgia, 30033, United States

Location

Riley Children's Hospital

Indianapolis, Indiana, 46202, United States

Location

Boston Children's Hospital

Boston, Massachusetts, 02111, United States

Location

University of Michigan Health System/Mott Hospital

Ann Arbor, Michigan, 48109, United States

Location

Children's Mercy Hospital

Kansas City, Missouri, 64108, United States

Location

Columbia College of Physicians and Surgeons

New York, New York, 10032, United States

Location

Cincinnati Children's Hospital Medical Center

Cincinnati, Ohio, 45229, United States

Location

Children's Hospital of Philadelphia

Philadelphia, Pennsylvania, 19104, United States

Location

Medical University of South Carolina

Charleston, South Carolina, 29425, United States

Location

Baylor College of Medicine - Texas Children's Hospital

Houston, Texas, 77030, United States

Location

Primary Children's Hospital

Salt Lake City, Utah, 84132, United States

Location

Hospital for Sick Children

Toronto, Ontario, M5G 1X8, Canada

Location

Biospecimen

Retention: SAMPLES WITH DNA

We aim to collect saliva samples from study participants during the single study visit at the PHN site, which will be preserved and processed by the PHN Biorepository for DNA storage for future targeted research studies.

MeSH Terms

Conditions

Down SyndromeHeart Defects, Congenital

Interventions

Reoperation

Condition Hierarchy (Ancestors)

Intellectual DisabilityNeurobehavioral ManifestationsNeurologic ManifestationsNervous System DiseasesAbnormalities, MultipleCongenital AbnormalitiesCongenital, Hereditary, and Neonatal Diseases and AbnormalitiesChromosome DisordersGenetic Diseases, InbornCardiovascular AbnormalitiesCardiovascular DiseasesHeart Diseases

Intervention Hierarchy (Ancestors)

Surgical Procedures, Operative

Study Officials

  • Maria VanRompay, PhD

    Carelon Research

    PRINCIPAL INVESTIGATOR

Study Design

Study Type
observational
Observational Model
CASE CONTROL
Time Perspective
PROSPECTIVE
Sponsor Type
OTHER
Responsible Party
SPONSOR

Study Record Dates

First Submitted

February 24, 2020

First Posted

April 5, 2022

Study Start

May 1, 2022

Primary Completion

December 31, 2023

Study Completion

December 31, 2023

Last Updated

January 11, 2024

Record last verified: 2023-10

Data Sharing

IPD Sharing
Will share

Public Use Datasets are created after study closeout and are made available on the PHN public website. Annotated study data collection forms, Statistical Analysis Program (SAS) datasets, and excel datasets are made available, as well as de-identified study cohort characteristics.

Shared Documents
STUDY PROTOCOL, ICF
Time Frame
Data is available one year after study closeout and is available indefinitely.
Access Criteria
log-in and password
More information

Locations