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Activated fibroblasts drive cellular interactions in end-stage pediatric hypertrophic cardiomyopathy

Hanna J Tadros1, Diwakar Turaga2,3, Yi Zhao4

  • 1Department of Pediatrics, Section of Pediatric Cardiology, Texas Children's Hospital, Baylor College of Medicine, Houston, TX, USA.

Biorxiv : the Preprint Server for Biology
|February 14, 2024
PubMed

Insights

Pediatric end-stage hypertrophic cardiomyopathy (HCM) shows distinct cellular changes, including stressed cardiomyocytes and increased fibrosis. This study offers the first single-nucleus analysis of pediatric HCM.

Area of Science:

  • Cardiovascular Biology
  • Pediatric Cardiology
  • Molecular Genetics

Background:

  • Hypertrophic cardiomyopathy (HCM) is a rare, debilitating pediatric diagnosis.
  • End-stage HCM necessitates heart transplantation.
  • Understanding pediatric HCM's cellular basis is crucial.

Approach:

  • Single-nucleus RNA sequencing (snRNA-seq) was performed on pediatric HCM and control myocardium.
  • Cellular processes in cardiomyocytes, fibroblasts, endothelial cells, and myeloid cells were analyzed.
  • Comparison between pediatric HCM and control samples provided insights.

Key Points:

  • Pediatric HCM cardiomyocytes showed "stressed" myocardium signatures and cardiac hypertrophy pathways.
  • Cardiac fibroblasts displayed heightened activation and fibrosis-associated processes, exceeding adult counterparts.
  • Tissue-resident macrophages were depleted, and endothelial cells showed increased vascular remodeling.

Conclusions:

  • This study presents the first single-nucleus analysis of end-stage pediatric HCM.
  • Distinct cellular and molecular alterations characterize pediatric HCM.
  • Findings highlight novel therapeutic targets for pediatric HCM.

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