NCT05312177

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

• Status: Completed
• Phase: —
• Study type: Observational
• Enrollment: 273 (actual)
• Sponsor: Carelon Research · Academic / Other
• Sex: All
• Age: 60 Months – 155 Months
• Healthy volunteers: Accepted

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).

Conditions

• Down Syndrome
• Congenital Heart Disease

Interventions

Type	Name	Description
OTHER	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.
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.
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.
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.
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).
OTHER	Social Communication Questionnaire, Current Form	The SCQ is a parent-report measure of social communication, social interactions, play, and behavior.
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.
OTHER	Repetitive Behavior Scale, Revised	The RBS-R is a parent report measure that comprehensively surveys for the presence of repetitive behaviors.

Timeline

• Start date: 2022-05-01
• Primary completion: 2023-12-31
• Completion: 2023-12-31
• First posted: 2022-04-05
• Last updated: 2024-01-11

Locations

14 sites across 2 countries: United States, Canada