The Many Faces of Arrhythmogenic Cardiomyopathy: An Overview

Authors

Hanna J Tadros
Christina Y Miyake
Debra L Kearney
Jeffrey J Kim
Susan W Denfield

Publication Date

1-1-2023

Journal

The Application of Clinical Genetics

DOI

10.2147/TACG.S383446

PMCID

37933265

PubMedCentral® Posted Date

11-1-2023

PubMedCentral® Full Text Version

Post-print

Published Open-Access

yes

Keywords

arrhythmias, arrhythmogenic cardiomyopathy, cardiomyopathy, genotype-to-phenotype correlation, pediatrics

Abstract

Arrhythmogenic cardiomyopathy (AC) is a disease that involves electromechanical uncoupling of cardiomyocytes. This leads to characteristic histologic changes that ultimately lead to the arrhythmogenic clinical features of the disease. Initially thought to affect the right ventricle predominantly, more recent data show that it can affect both the ventricles or the left ventricle alone. Throughout the recent era, diagnostic modalities and criteria for AC have continued to evolve and our understanding of its clinical features in different age groups as well as the genotype to the phenotype correlations have improved. In this review, we set out to detail the epidemiology, etiologies, presentations, evaluation, and management of AC across the age continuum.