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

Carelon Research273 enrolled

Overview

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.

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 Type

OBSERVATIONAL

Enrollment

273

Conditions

Down Syndrome Congenital Heart Disease

Interventions

Stanford Binet Intelligence Scales, Fifth EditionOTHER

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 EditionOTHER

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.

Eligibility

Sex: ALLMin age: 60 MonthsMax age: 155 MonthsHealthy volunteers:

Medical Language ↔ Plain English

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.

Locations (14)

Alfred I. duPont Hospital for Children

Wilmington, Delaware, United States

Children's National Hospital

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

Outcomes

Primary Outcomes

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.

Time frame: 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.

Time frame: 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.

Time frame: 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.

Time frame: 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.

Time frame: 1 day

Data from ClinicalTrials.gov

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

Overview

Conditions

Interventions

Eligibility

Locations (14)

Outcomes

Primary Outcomes

Secondary Outcomes