# Impact of Congenital Heart Disease Surgery on Neurodevelopment and Behavior in Children with Down Syndrome - Admin Supp

> **NIH NIH U24** · NEW ENGLAND RESEARCH INSTITUTES, INC. · 2021 · $308,234

## Abstract

PROJECT SUMMARY/ABSTRACT
Congenital heart disease (CHD) affects 40-50% of individuals with Down syndrome (DS), most commonly
complete atrioventricular septal defect (CAVSD). Although the impact of perioperative risk factors during infant
heart surgery are well studied in the general population, DS patients have been excluded from virtually all
prospective studies on determinants of neurodevelopmental (ND) outcomes after infant heart surgery. Our
overarching goal is to analyze the impact of CHD repaired in infancy on overall ND and behavioral outcomes in
the DS population. Our proposed study is ancillary to the NHLBI-funded, prospective, Pediatric Heart Network
(PHN) Residual Lesion Score (RLS) Study, which enrolled 207 DS patients with CAVSD. The parent RLS
Study is evaluating the effect of technical adequacy of repair after infant heart surgery on early and mid-term
outcomes, including perioperative morbidity and mortality, with follow-up through 12 months. Here, we will
investigate determinants of ND and behavioral outcomes at 3-5 years of age in the RLS Study sample with DS
who had CAVSD repair in the first year of life compared to an age-matched sample with DS without CHD from
the same PHN sites. In Aim 1 (primary), we will compare ND and behavioral outcomes between children with
DS who had CAVSD repair and children from the same clinical sites with DS without CHD. Primary outcomes
will be (i) verbal and non-verbal ratio IQs derived from the Mullen Scales of Early Learning and (ii) adaptive
composite scores from the Vineland Adaptive Behavior Scales-3rd edition, and secondary outcomes will be (i)
language scores derived from the Preschool Language Scales-5th edition, (ii) emotional and behavioral
functioning scores on the Childhood Behavior Checklist, and (iii) attention and executive functioning scores on
the Behavior Rating Inventory of Executive Functioning, Preschool Version. In Aim 2, we will investigate
predictors of ND and behavior as measured in Aim 1 in children with DS with CAVSD repair and those without
CHD, utilizing structural equation modeling to explore relationships and possible mediation among predictor
variables. In Aim 3, we will establish a collection of biological material for future studies exploring genetic
factors contributing to the wide spectrum of ND abilities seen in DS. We will incorporate a parent advisory
group for research questions, design of study materials, and dissemination to the DS community. In addition to
creating a large cohort of children with DS and CAVSD repair and an age-matched comparison group, this
study addresses an important knowledge gap about whether CHD requiring infant heart surgery plays an
important role in ND and behavioral outcomes in children with DS. If so, targeted interventions beginning in the
early postoperative period could be designed for this vulnerable population. Positive or negative, this study will
provide invaluable data for prenatal counseling on the impact of CAVSD on l...

## Key facts

- **NIH application ID:** 10409121
- **Project number:** 3U24HL135691-04S1
- **Recipient organization:** NEW ENGLAND RESEARCH INSTITUTES, INC.
- **Principal Investigator:** Nicole Baumer
- **Activity code:** U24 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 2021
- **Award amount:** $308,234
- **Award type:** 3
- **Project period:** 2021-07-01 → 2024-12-31

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/10409121

## Citation

> US National Institutes of Health, RePORTER application 10409121, Impact of Congenital Heart Disease Surgery on Neurodevelopment and Behavior in Children with Down Syndrome - Admin Supp (3U24HL135691-04S1). Retrieved via AI Analytics 2026-05-23 from https://api.ai-analytics.org/grant/nih/10409121. Licensed CC0.

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