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Arrhythmogenic right ventricular cardiomyopathy: Treatment and prognosis

Author
William J McKenna, MD
Section Editor
Hugh Calkins, MD
Deputy Editor
Brian C Downey, MD, FACC

INTRODUCTION

Arrhythmogenic right ventricular cardiomyopathy (ARVC), also called arrhythmogenic right ventricular dysplasia (ARVD), is an underrecognized clinical entity manifested by ventricular arrhythmias and a specific ventricular pathology [1-3]. It is characterized macroscopically by a fatty appearance of the right ventricular (RV) free wall. The fibrofatty replacement of the RV myocardium initially produces typical regional wall motion abnormalities that later become global, producing RV dilation. The tissue replacement can also involve areas of the left ventricle (LV) with relative sparing of the septum [4].

The prevalence in the general population is estimated to be approximately 1:1000 [1]. ARVC appears to be relatively common in young adults in northern Italy [2,5], accounting for approximately 11 percent of the cases of sudden cardiac death (SCD) overall and 22 percent in athletes [5].

In contrast, ARVC has been rarely diagnosed in the United States, but it appears to be identified with increasing frequency. This may reflect a difference in genetic prevalence, but is more likely due to under recognition of disease. In a series of 100 ARVC patients from a single referral center in the United States, clinical profiles and event rates were similar to those reported in Europe [6].

The treatment and prognosis of ARVC will be reviewed here. The genetics, pathogenesis, clinical manifestations, diagnostic criteria, and evaluation are discussed separately. (See "Arrhythmogenic right ventricular cardiomyopathy: Pathogenesis and genetics" and "Arrhythmogenic right ventricular cardiomyopathy: Anatomy, histology, and clinical manifestations".)

TREATMENT

General therapeutic approach — The optimal strategies for preventing sudden cardiac death (SCD) and the indications for implantable cardioverter-defibrillator (ICD) therapy in patients with ARVC are the subject of international consensus documents [7-9]. Although some studies suggest that patients with hemodynamically tolerated arrhythmias do well when treated with antiarrhythmic drugs, guidelines recommend ICD implantation for secondary prevention of SCD in patients with sustained ventricular tachycardia (VT) or ventricular fibrillation (VF) and for primary prevention in selected high-risk patients [8,9]. Antiarrhythmic drugs may be required in patients with asymptomatic or symptomatic arrhythmia or as an adjunct to an ICD. (See 'Treatment of arrhythmia' below and 'Antiarrhythmic drugs' below.)

                  

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Literature review current through: Nov 2016. | This topic last updated: Thu Nov 17 00:00:00 GMT 2016.
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