Résumé
The arrhythmogenic right ventricular dysplasia (RVD)is an important cause of sudden death in young people, specially during strenuous exercise. It could be more frecuent than the hypertrophic cardiomyopathy. RVD could provoke sudden death also in apparently normal subjects due to ventricular tachyarrhythmias and/or complete AV block. In some cases RVD could progress very soon to dilated cardiomyopathy and congestive heart failure. The implant of an ICD is strongly suggested in patients resuscitated from a cardiac arrest episode, whose malignant arrhythmias are either non inducible or partially controlled with antiarrhythmic drugs during the electrophysiological study. The risk of these patients can be controlled with good long-term prognosis, with pharmacologic and non-pharmacologic therapies