The Many Faces of Arrhythmogenic Cardiomyopathy: An Overview

Abstract

Hanna J Tadros,1 Christina Y Miyake,1,2 Debra L Kearney,3 Jeffrey J Kim,1 Susan W Denfield4 1Department of Pediatrics, Section of Pediatric Cardiology, Texas Children’s Hospital, Baylor College of Medicine, Houston, TX, USA; 2Department of Molecular Physiology and Biophysics, Baylor College of Medicine, Houston, TX, USA; 3Department of Pathology, Texas Children’s Hospital, Baylor College of Medicine, Houston, TX, USA; 4Department of Pediatrics, Division of Pediatric Cardiology, Baylor College of Medicine, Houston, TX, USACorrespondence: Susan W Denfield, Baylor College of Medicine, One Baylor Plaza, Houston, TX, USA, Email [email protected]: 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.Keywords: arrhythmias, arrhythmogenic cardiomyopathy, cardiomyopathy, genotype-to-phenotype correlation, pediatrics

Keywords

• arrhythmias
• arrhythmogenic cardiomyopathy
• cardiomyopathy
• genotype-to-phenotype correlation
• pediatrics.