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

Shanshan Gao; Suet Nee Chen; Carlo Di Nardo; Raffaella Lombardi

doi:10.3389/fphys.2020.00834

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

Front Physiol. 2020 Jul 30:11:834. doi: 10.3389/fphys.2020.00834. eCollection 2020.

Authors

Shanshan Gao¹, Suet Nee Chen¹, Carlo Di Nardo², Raffaella Lombardi^{1

2}

Affiliations

¹ Division of Cardiology, Department of Medicine, University of Colorado, Aurora, CO, United States.
² Division of Cardiology, Department of Advanced Biomedical Sciences, Federico II University of Naples, Naples, Italy.

Abstract

Arrhythmogenic cardiomyopathy (ACM) is a heritable cardiac disease characterized by fibrotic or fibrofatty myocardial replacement, associated with an increased risk of ventricular arrhythmias and sudden cardiac death. Originally described as a disease of the right ventricle, ACM is currently recognized as a biventricular entity, due to the increasing numbers of reports of predominant left ventricular or biventricular involvement. Research over the last 20 years has significantly advanced our knowledge of the etiology and pathogenesis of ACM. Several etiopathogenetic theories have been proposed; among them, the most attractive one is the dystrophic theory, based on the observation of similar histopathological features between ACM and skeletal muscle dystrophies (SMDs), such as progressive muscular degeneration, inflammation, and tissue replacement by fatty and fibrous tissue. This review will describe the pathophysiological and molecular similarities shared by ACM with SMDs.

Keywords: arrhythmogenic cardiomyopathy; fibroadiposis; inflammation; molecular pathogenesis; skeletal muscle dystrophies.

Publication types

Review