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Arrhythmogenic Cardiomyopathy and Skeletal Muscle Dystrophies: Shared Histopathological Features and Pathogenic

Shanshan Gao1, Suet Nee Chen1, Carlo Di Nardo2

  • 1Division of Cardiology, Department of Medicine, University of Colorado, Aurora, CO, United States.

Frontiers in Physiology
|August 28, 2020
PubMed

Insights

Arrhythmogenic cardiomyopathy (ACM) shares similarities with skeletal muscle dystrophies (SMDs). This review explores the pathophysiological and molecular links between these heritable diseases.

Area of Science:

  • Cardiology
  • Genetics
  • Pathology

Background:

  • Arrhythmogenic cardiomyopathy (ACM) is an inherited heart condition.
  • It involves fibrotic or fibrofatty tissue replacing heart muscle.
  • ACM increases the risk of sudden cardiac death and arrhythmias.

Purpose of the Study:

  • To review the pathophysiological and molecular similarities between ACM and skeletal muscle dystrophies (SMDs).
  • To explore the 'dystrophic theory' of ACM.
  • To advance understanding of ACM's origins and progression.

Main Methods:

  • Literature review of research on ACM and SMDs.
  • Analysis of histopathological and molecular data.
  • Comparison of disease mechanisms and features.

Main Results:

  • ACM is increasingly recognized as a biventricular disease, not just right ventricular.
  • Histopathological similarities exist between ACM and SMDs.
  • These include progressive degeneration, inflammation, and tissue replacement.

Conclusions:

  • The dystrophic theory provides a compelling framework for understanding ACM.
  • Shared molecular and pathophysiological pathways may link ACM and SMDs.
  • Further research into these similarities could reveal new therapeutic targets.

Keywords:
arrhythmogenic cardiomyopathyfibroadiposisinflammationmolecular pathogenesisskeletal muscle dystrophies

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