Journal of Heart and Cardiology | 2016-04-26

Arrhythmogenic right ventricular dysplasia / THE DARK VOYAGE OF THE FEARLESS EAGLE /Case presentation and review of the literature

Samer Nasr

Abstract

Arrhythmogenic right ventricular dysplasia (ARVD) is an obscure clinical entity, due to its infrequent presentation, difficulty in diagnosis and the outcome of missing it could be lethal. Inherited by transmission of affected desmosomal and characterized by the replacement of the myocardial cell with fibrofatty tissue, leading to ventricular arrhythmias. Regional wall motion abnormalities are the earliest signs of right ventricle (RV) myocardial scarring that will progress later to global injury leading to RV dilation and failure, with sudden cardiac death (SCD) in young adults as major end point in the course of this disease. Although it mainly affects the RV, some variants of ARVD can also affect the left ventricle with or without involvement of the septum. The aim of this article is a quick review on ARVD, how to recognize the clinical manifestation, how to diagnose it and how to approach relatives to SCD victims.

Keywords: cardiomyopathy, diagnosis, echocardiography, electrocardiography, magnetic resonance imaging, approach