IMR Press / RCM / Volume 22 / Issue 4 / DOI: 10.31083/j.rcm2204136
Open Access Review
Arrhythmogenic right ventricular cardiomyopathy: diverse substrate characteristics and ablation outcome
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1 Heart Rhythm Center and Division of Cardiology, Department of Medicine, Taipei Veterans General Hospital, 112201 Taipei, Taiwan
2 Department of Medicine, School of Medicine, National Yang Ming Chiao Tung University, 112304 Taipei, Taiwan
3 Department of Medicine, Taipei Veterans General Hospital Taitung Branch, 95050 Taitung, Taiwan
4 Cardiovascular Center, Taichung Veterans General Hospital, 40705 Taichung, Taiwan
*Correspondence: (Fa-Po Chung)
These authors contributed equally.
Academic Editors: Brian Tomlinson and Takatoshi Kasai
Rev. Cardiovasc. Med. 2021, 22(4), 1295–1309;
Submitted: 26 August 2021 | Revised: 20 October 2021 | Accepted: 20 October 2021 | Published: 22 December 2021
(This article belongs to the Special Issue State-of-the-Art Cardiovascular Medicine in Asia 2021)
Copyright: © 2021 The Author(s). Published by IMR Press.
This is an open access article under the CC BY 4.0 license (

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy caused by defective desmosomal proteins. The typical histopathological finding of ARVC is characterized by progressive fibrofatty infiltration of the right ventricle due to the dysfunction of cellular adhesion molecules, thus, developing arrhythmogenic substrates responsible for the clinical manifestation of ventricular tachycardia/fibrillation (VT/VF). Current guidelines recommend implantable cardiac defibrillator (ICD) implantation to prevent sudden cardiac death (SCD) in ARVC, especially for those experiencing VT/VF or aborted SCD, while antiarrhythmic drugs, despite their modest effectiveness and several undesirable adverse effects, are frequently used for those experiencing episodes of ICD interventions. Given the advances in mapping and ablation technologies, catheter ablation has been implemented to eliminate drug-refractory VT in ARVC. A better understanding of the pathogenesis, underlying arrhythmogenic substrates, and putative VT isthmus in ARVC contributes to a significant improvement in ablation outcomes through comprehensive endocardial and epicardial approaches. Regardless of ablation strategies, there is a diversity of arrhythmogenic substrates in ARVC, which could partly explain the nonuniform ablation outcome and long-term recurrences and reflect the role of potential factors in the modification of disease progression and triggering of arrhythmic events.

Arrhythmogenic right ventricular cardiomyopathy
Epicardial approach
Ventricular fibrillation
Ventricular tachycardia
Fig. 1.
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