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Myocardial Fibrosis in Hypertrophic Cardiomyopathy: A Perspective from Fibroblasts

Maja Schlittler1, Peter P Pramstaller1, Alessandra Rossini1

  • 1Eurac Research, Institute for Biomedicine (Affiliated to the University of Lübeck), 39100 Bolzano, Italy.

International Journal of Molecular Sciences
|October 14, 2023
PubMed

Insights

Hypertrophic cardiomyopathy (HCM), an inherited heart condition, involves cardiomyocyte hypertrophy and fibrosis. This review explores fibrosis promotion, fibroblast roles, and TGF-β signaling in HCM pathophysiology.

Area of Science:

  • Cardiovascular Medicine
  • Genetics
  • Molecular Biology

Background:

  • Hypertrophic cardiomyopathy (HCM) is the most common inherited heart disease and a leading cause of sudden cardiac death in young individuals.
  • Genetic mutations in cardiac sarcomere proteins are frequent causes of HCM.
  • HCM is characterized by cardiomyocyte hypertrophy and myocardial fibrosis, impacting cardiac function.

Purpose of the Study:

  • To review the latest evidence on the mechanisms promoting cardiac fibrosis in HCM.
  • To elucidate the role of cardiac fibroblasts and their interaction with cardiomyocytes.
  • To summarize findings on profibrotic genes and genetic/non-genetic factors in HCM pathophysiology.

Main Methods:

  • Literature review of recent evidence on cardiac fibrosis in HCM.
  • Analysis of the role of cardiac fibroblasts and their activation pathways.
  • Summary of genetic and non-genetic factors contributing to HCM.

Main Results:

  • Cardiac fibrosis, excessive deposition of collagen I and III, adversely affects heart function.
  • The TGF-β pathway is a primary regulator of extracellular matrix turnover and fibroblast activation.
  • New profibrotic genes and contributing factors to HCM are identified.

Conclusions:

  • Understanding fibrosis promotion, fibroblast activation, and TGF-β signaling is crucial for HCM.
  • Identifying genetic and non-genetic factors advances knowledge of HCM pathophysiology.
  • This review synthesizes current knowledge on HCM, focusing on fibrotic mechanisms.

Keywords:
TGF-βcardiac fibroblasthypertrophic cardiomyopathymyocardial fibrosismyofibroblasts

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