Arrhythmogenic Right Ventricular Dysplasia: cardiomyopathy current opinions on diagnostic and therapeutic aspects

Right Ventricular Dysplasia constitutes a genetic cardiomyopathy characteri zed by fibrous-adipose substitution of the right and rarely of the left ven tricular myocardium. This disorder is associated with ventricular arrhythmi as ranging from frequent ventricular ectopic beats, nonsustained and sustai ned ventricular tachycardia of left bundle branch morphology and sudden dea th, Therefore, the syndrome has been labelled Arrhythmogenic RVD Cardiomyop athy. Diagnostic criteria, preliminary genetic data, and clinical manifestations are summarized and critical addressed, using data from the literature and f rom our own experience. The most important aspects of the ECG in this syndr ome are reviewed and stressed with particular attention to initial versus a dvanced clinical subsets. The typical anatomical abnormalities and biopsy o r pathology material are presented. Curr Opin Cardiol 2001, 16:8-16 (C) 200 1 Lippincott Williams & Wilkins, Inc.