Arrhythmogenic right ventricular dysplasia

Abstract

The arrhythmogenic right ventricular dysplasia is a genetic base cardiomyopathy, characterizedby the substitution of the miocardic tissue by an adipose or a fibroadipose one. Itusually affects the right ventricle, but the septum and the left ventricle can also be compromise.It is more frequent in young men and athletes. The most common clinical manifestationare ventricular arrhythmias and frequently the sudden death is the first event. In advancedstages systolic biventricular dysfunction exists. The electrocardiogram is characterizedby alterations in despolarization and in ventricular repolarization in right precordial derivations.The most validated image techniques for their diagnosis are the ecocardiogram,the ventriculography and the nuclear magnetic resonance, these might show global dilationof the right ventricle with systolic disfuntion, segmental wall motility dysfunctions and aneurismsof the wall. The dysplasia risk stratification is guided to prevent the arrhythmic sudden death. The therapeutic options include beta-blocking, group III antiarrhytmic drugs, automaticimplantable desfibrillator, radiofrecuency ablation and heart transplant. The presentreview has the objective to modernize the concepts of the entity, from the genetic, diagnostic,prognostic and therapeutic point of view.

Keywords

• Sudden death
• arrhythmogenic right ventricular dysplasia
• ventricular arrhythmias.