Arrhythmogenic Right Ventricular Dysplasia

Abstract

A rrhythmogenic right ventricular dysplasia (ARVD), which was first described in 1977, is a poorly understood yet potentially lethal cause of cardiac disease. 1,2 Once thought to be rare, ARVD has been shown to have an incidence of six per 10,000 persons in certain populations. 3 In certain Mediterranean and southern U.S. populations, the incidence is as high as 44 per 10,000 persons. 3 ARVD accounts for 3 to 4 percent of deaths in sports and 5 percent of sudden cardiac deaths in persons younger than 65 years. 4-7 Given the relative frequency of this disorder and its potential for disastrous outcomes, it is important for primary care physicians to be familiar with its presentation, diagnosis, and management.

Electrocardiographic Features of Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy According to Disease Severity

“…3 These criteria included (1) T-wave inversions in V 1 through V 3 , (2) QRS duration (QRSd) Ն110 ms in V 1 through V 3 , and (3) the presence of an epsilon wave (electric potentials after the end of the QRS complex). Additional ECG markers of ARVD/C that have been reported include (1) QRS and QT dispersion, 4 (2) parietal block, defined as a QRSd in leads V 1 through V 3 that exceeds the QRSd in lead V 6 by Ͼ25 ms, 5 and (3) a ratio of the QRSd in leads V 1 ϩV 2 ϩV 3 /V 4 ϩV 5 ϩV 6 Ն1.2. 6 The purpose of the present study was to reexamine the ECG features of ARVD/C.…”

Mutations in the desmosomal protein plakophilin-2 are common in arrhythmogenic right ventricular cardiomyopathy

“…Individuals with ARVC may present with palpitations or ventricular tachyarrhythmias that originate from the right ventricle and may lead to syncope or sudden cardiac death, a relatively common and the most feared clinical manifestation of the disease [1][2][3] . Localized or diffuse atrophy and progressive replacement of right ventricular myocytes with fatty or fibrofatty tissue is the pathological hallmark of ARVC.…”

Arrhythmogenic right ventricular cardiomyopathy/dysplasia: a not so rare “disease of the desmosome” with multiple clinical presentations

“…Very rarely, patients may complain of chest discomfort or anginal pain on exertion. The patients are usually young (33 ±14 years, range 13-73 15 ), with a male predominance. In patients with less arrhythmogenic forms of the disease, symptoms and signs of heart failure may be the first presentation.…”

“…The value of signal averaged ECG in ARVC/D is controversial 15 . It is used to detect delayed afterdepolarizations ("late potentials").…”

“…The sensitivity was reported to be 67% with a specificity of 92% 15 . Often, the interventricular septum is not involved in the disease, and sampling errors may occur.…”