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

Completed

• Conditions: Congenital Heart Disease; Down Syndrome

• Registration Number: NCT05312177
• Lead Sponsor: Carelon Research

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.

Detailed Description

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).

Study Timeline

• Study First Submitted: 2020-02-24
• Study First Submitted QC: 2022-03-31
• Study First Posted: 2022-04-05
• Study Start: 2022-05-01
• Status Verified: 2023-10
• Primary Completion: 2023-12-31
• Study Completion: 2023-12-31
• Last Update Submitted: 2024-01-10
• Last Update Posted: 2024-01-11

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 273

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.

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
Behavioral outcomes between children with DS who had CAVSD repair and children from the same clinical sites with DS without major CHD.	1 day	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.
Social Communication compared between the children with DS and CAVSD repair and the children with DS without major CHD.	1 day	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.
Language abilities compared between the children with DS and CAVSD repair and the children with DS without major CHD.	1 day	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.
Neurodevelopmental outcomes between children with DS who had CAVSD repair and children from the same clinical sites with DS without major CHD.	1 day	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.
Emotional outcomes compared between the children with DS and CAVSD repair and the children with DS without major CHD.	1 day	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.

Secondary Outcome Measures

Name	Time	Method
Sociodemographic factors as predictors of neurodevelopment and behavior	1 day	Sociodemographic factors, such as will be compared between the children with DS and CAVSD repair and the children with DS without major CHD.
Comorbidities as predictors of neurodevelopment and behavior	1 day	Comorbidities will be compared between the children with DS and CAVSD repair and the children with DS without major CHD.

Trial Locations

Locations (14)

Alfred I. duPont Hospital for Children; 🇺🇸 Wilmington, Delaware, United States

Children's National Hospital; 🇺🇸 Washington, District of Columbia, United States

Children's Healthcare of Atlanta at Egleston; 🇺🇸 Atlanta, Georgia, United States

Riley Children's Hospital; 🇺🇸 Indianapolis, Indiana, United States

Boston Children's Hospital; 🇺🇸 Boston, Massachusetts, United States

University of Michigan Health System/Mott Hospital; 🇺🇸 Ann Arbor, Michigan, United States

Children's Mercy Hospital; 🇺🇸 Kansas City, Missouri, United States

Columbia College of Physicians and Surgeons; 🇺🇸 New York, New York, United States

Cincinnati Children's Hospital Medical Center; 🇺🇸 Cincinnati, Ohio, United States

Children's Hospital of Philadelphia; 🇺🇸 Philadelphia, Pennsylvania, United States

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