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Current understanding of fibrosis in genetic cardiomyopathies

Tim R Eijgenraam1, Herman H W Silljé1, Rudolf A de Boer1

  • 1Department of Cardiology, University Medical Center Groningen, University of Groningen, Antonius Deusinglaan 1, 9713 AV Groningen, the Netherlands.

Trends in Cardiovascular Medicine
|October 6, 2019
PubMed

Insights

Myocardial fibrosis, excessive heart scarring, is a hallmark of cardiomyopathies and linked to adverse outcomes. New non-invasive imaging techniques help visualize this fibrosis in genetic heart conditions.

Area of Science:

  • Cardiology
  • Pathology
  • Biomedical Imaging

Background:

  • Myocardial fibrosis involves excessive deposition of extracellular matrix proteins in the heart.
  • It is a key feature of pathological cardiac remodeling in various cardiomyopathies.
  • While initially reparative, progressive fibrosis can lead to heart failure and arrhythmias.

Purpose of the Study:

  • To review the epidemiology, distribution, and role of myocardial fibrosis in genetic cardiomyopathies.
  • To highlight characteristic fibrosis patterns in different genetic cardiomyopathy subtypes.
  • To discuss the clinical implications of myocardial fibrosis in these conditions.

Main Methods:

  • Literature review focusing on myocardial fibrosis in genetic cardiomyopathies.
  • Analysis of epidemiological data and fibrosis distribution patterns.
  • Evaluation of emerging non-invasive in vivo imaging techniques for fibrosis visualization.

Main Results:

  • Myocardial fibrosis patterns serve as distinctive hallmarks for specific genetic cardiomyopathies.
  • The presence of fibrosis, even subtle, correlates with complications and adverse outcomes.
  • Non-invasive imaging techniques are increasingly utilized in research and clinical settings.

Conclusions:

  • Myocardial fibrosis is a critical factor in the progression and prognosis of genetic cardiomyopathies.
  • Understanding fibrosis patterns aids in diagnosis and risk stratification.
  • Advancements in imaging offer new avenues for studying and managing myocardial fibrosis.

Keywords:
CardiomyopathyFibrosisGeneticsHeart failureImagingRemodeling

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