Arrhythmogenic cardiomyopathy (ACM) is an inherited heart disorder, predisposing to malignant ventricular arrhythmias leading to sudden cardiac death, particularly in young and athletic patients. Pathological features include a progressive loss of myocardium with fibrous or fibro-fatty substitution. During the last few decades, different clinical aspects of ACM have been well investigated but still little is known about the molecular mechanisms that underlie ACM pathogenesis, leading to these phenotypes. In about 50% of ACM patients, a genetic mutation, predominantly in genes that encode for desmosomal proteins, has been identified. However, the mutation-associated mechanisms, causing the observed cardiac phenotype are not always clear. Until now, the attention has been principally focused on the study of molecular mechanisms that lead to a prominent myocardium adipose substitution, an uncommon marker for a cardiac disease, thus often recognized as hallmark of ACM. Nonetheless, based on Task Force Criteria for the diagnosis of ACM, cardiomyocytes death associated with fibrous replacement of the ventricular free wall must be considered the main tissue feature in ACM patients. For this reason, it urges to investigate ACM cardiac fibrosis. In this review, we give an overview on the cellular effectors, possible triggers, and molecular mechanisms that could be responsible for the ventricular fibrotic remodeling in ACM patients.
Fibrosis in Arrhythmogenic Cardiomyopathy: The Phantom Thread in the Fibro-Adipose Tissue / A.S. Maione, C.A. Pilato, M. Casella, A. Gasperetti, I. Stadiotti, G. Pompilio, E. Sommariva. - In: FRONTIERS IN PHYSIOLOGY. - ISSN 1664-042X. - 11(2020 Apr 03).
|Titolo:||Fibrosis in Arrhythmogenic Cardiomyopathy: The Phantom Thread in the Fibro-Adipose Tissue|
POMPILIO, GIULIO (Penultimo)
|Parole Chiave:||arrhythmogenic cardiomyopathy; cardiac extracellular matrix; cardiac fibrosis; cellular effectors; scar formation|
|Settore Scientifico Disciplinare:||Settore MED/11 - Malattie dell'Apparato Cardiovascolare|
Settore MED/23 - Chirurgia Cardiaca
|Data di pubblicazione:||3-apr-2020|
|Digital Object Identifier (DOI):||http://dx.doi.org/10.3389/fphys.2020.00279|
|Appare nelle tipologie:||01 - Articolo su periodico|