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Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia

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Electrical Diseases of the Heart

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Abstract

Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia (ARVC/D) is an inherited ­cardiomyopathy characterized clinically by ventricular arrhythmias, sudden death, and structural abnormalities of the right ventricle. Although structural involvement of the right ventricle predominates, a left dominant form (LDAC) has been described. Structural involvement of both ventricles is common in advanced disease. Due to significant heterogeneity in its manifestation, the diagnosis of ARVC/D is challenging and requires a multifaceted approach to patient evaluation. The recently published Revised Task Force Criteria provide clinicians with a validated model to improve the diagnosis of ARVC/D. The management of ARVC/D is primarily aimed at reducing the burden of symptomatic arrhythmias and decreasing the incidence of sudden ­cardiac death. Automatic implantable cardioverter-defibrillators (AICDs) significantly reduce mortality in patients with ARVC/D. However, accurate risk assessment is needed to avoid exposing low-risk patients to the long-term complications of AICDs. Strenuous exertion increases the rate of disease progression and increases the risk of sudden death.

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Author information

Authors and Affiliations

  1. Department of Cardiology, School of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, USA

    Hugh Calkins MD

  2. Department of Cardiology, The Johns Hopkins Hospital, 1800 Orleans Street, Baltimore, 21287, MD, USA

    Hugh Calkins MD

  3. Division of Cardiology, The University of Arizona, Sarver Heart Center, Tucson, AZ, USA

    Frank Marcus MD

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  1. Hugh Calkins MD
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  2. Frank Marcus MD
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Correspondence to Hugh Calkins MD .

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Editors and Affiliations

  1. , Department of Internal Medicine, UMDNJ-RWJ Medical School, Robert Wood Johnson Pl. 1, New Brunswick, 08903-0019, New Jersey, USA

    Ihor Gussak

  2. , Gordon K. Moe Scholar, Masonic Medical Research Laboratory, Bleecker Street, Utica, 13501-1787, New York, USA

    Charles Antzelevitch

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© 2013 Springer-Verlag London

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Calkins, H., Marcus, F. (2013). Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia. In: Gussak, I., Antzelevitch, C. (eds) Electrical Diseases of the Heart. Springer, London. https://doi.org/10.1007/978-1-4471-4978-1_4

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  • DOI: https://doi.org/10.1007/978-1-4471-4978-1_4

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