Arrhythmogenic Right Ventricular Cardiomyopathy: A Comprehensive Review

Authors

Taha Shaikh, University of Nevada, Las VegasFollow
Darren Nguyen, University of Nevada, Las VegasFollow
Jasmine Dugal, University of Nevada, Las VegasFollow
Michael DiCaro, University of Nevada, Las VegasFollow
Brianna Yee, University of Nevada, Las VegasFollow
Nazanin Houshmand, University of Nevada, Las VegasFollow
KaChon Lei, University of Nevada, Las VegasFollow
Ali Namazi, University of Nevada, Las VegasFollow

Document Type

Article

Publication Date

2-13-2025

Publication Title

Journal of Cardiovascular Development and Disease

Volume

12

Issue

2

First page number:

1

Last page number:

25

Abstract

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by structural abnormalities, arrhythmias, and a spectrum of genetic and clinical manifestations. Clinically, ARVC is structurally distinguished by right ventricular dilation due to increased adiposity and fibrosis in the ventricular walls, and it manifests as cardiac arrhythmias ranging from non-sustained ventricular tachycardia to sudden cardiac death. Its prevalence has been estimated to range from 1 in every 1000 to 5000 people, with its large range being attributed to the variability in genetic penetrance from asymptomatic to significant burden. It is even suggested that the prevalence is underestimated, as the presence of genotypic mutations does not always lead to clinical manifestations that would facilitate diagnosis. Additionally, while set criteria have been in place since the 1990s, newer understanding of this condition and advancements in cardiac technology have prompted multiple revisions in the diagnostic criteria for ARVC. Novel discoveries of gene variants predisposing patients to ARVC have led to established screening techniques while providing insight into genetic counseling and management. This review aims to provide an overview of the genetics, pathophysiology, and clinical approach to ARVC. It will also focus on clinical presentation, ARVC diagnostic criteria, electrophysiological findings, including electrocardiogram characteristics, and imaging findings from cardiac MRI, 2D, and 3D echocardiogram. Current management options—including anti-arrhythmic medications, device indications, and ablation techniques—and the effectiveness of treatment will also be reviewed.

Keywords

arrhythmogenic; heart disease; cardiovascular disease; cardiomyopathy; ARVC; VT; ventricular tachycardia; electrophysiology; congenital

Disciplines

Cardiovascular Diseases | Cardiovascular System | Disease Modeling

File Format

PDF

File Size

4900 KB

Language

English

Rights

IN COPYRIGHT. For more information about this rights statement, please visit http://rightsstatements.org/vocab/InC/1.0/

Creative Commons License

This work is licensed under a Creative Commons Attribution 4.0 International License.

Repository Citation

Shaikh, T., Nguyen, D., Dugal, J., DiCaro, M., Yee, B., Houshmand, N., Lei, K., Namazi, A. (2025). Arrhythmogenic Right Ventricular Cardiomyopathy: A Comprehensive Review. Journal of Cardiovascular Development and Disease, 12(2), 1-25.
Available at: http://dx.doi.org/10.3390/jcdd12020071