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Clinical Cases in Right Heart Failure pp 93–123Cite as

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC)

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Part of the book series: Clinical Cases in Cardiology ((CCC))

Abstract

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic cardiomyopathy caused by pathogenic mutations in genes encoding protein components of intercalated discs, such as desmosome. This results in impaired intercalated disc function, cardiomyocyte loss, and fibrofatty replacement. Patients with ARVC commonly present with ventricular arrhythmias. Most patients do not have heart failure symptoms early in the course of the disease. As the disease progresses, symptoms of heart failure, especially right-sided (fatigue, exertional intolerance, lower extremity edema, and abdominal bloating), may subsequently develop from progressive contractile dysfunction and dilatation of the right ventricle. ARVC is clinically diagnosed by a set of criteria incorporating ECG, imaging, and genetic findings. Implantable cardioverter-defibrillator (ICD) implantation and avoiding high intensity endurance exercise plays a central role in reducing the risk of sudden cardiac death. The role of medications, including antiarrhythmic drugs, is largely for symptomatic treatment.

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Authors and Affiliations

  1. Division of Cardiology, Department of Internal Medicine, UT Southwestern Medical Center, Dallas, TX, USA

    Nath Zungsontiporn & Mark S. Link

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  1. Nath Zungsontiporn
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  2. Mark S. Link
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Correspondence to Mark S. Link .

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Editors and Affiliations

  1. St. Elizabeth’s Medical Center, Boston, MA, USA

    Lana Tsao

  2. Maine Medical Center, Portland, ME, USA

    Maxwell E. Afari

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Zungsontiporn, N., Link, M.S. (2020). Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC). In: Tsao, L., Afari, M. (eds) Clinical Cases in Right Heart Failure. Clinical Cases in Cardiology. Springer, Cham. https://doi.org/10.1007/978-3-030-38662-7_5

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  • DOI: https://doi.org/10.1007/978-3-030-38662-7_5

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