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Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia

Chapter

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.

Keywords

Arrhythmogenic Right Ventricular Dysplasia (ARVD) Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC/D) Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy (ARVD/C) Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia (ARVC/D) Arrhythmogenic Cardiomyopathy 

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Copyright information

© Springer-Verlag London 2013

Authors and Affiliations

  1. 1.Department of CardiologySchool of Medicine, Johns Hopkins University School of MedicineBaltimoreUSA
  2. 2.Department of CardiologyThe Johns Hopkins HospitalBaltimoreUSA
  3. 3.Division of CardiologyThe University of Arizona, Sarver Heart CenterTucsonUSA

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