World Journal of Cardiovascular Diseases, 2015, 5, 218-226 Published Online August 2015 in SciRes. http://www.scirp.org/journal/wjcd http://dx.doi.org/10.4236/wjcd.2015.58025 How to cite this paper: Bonny, A., Talle, M.A. and Fontaine, G. (2015) Arrhythmogenic Ventricular Dysplasia/Cardiomyo- pathy: Insights from the Rationale of Disease Nomenclature and Clinical Perspectives. World Journal of Cardiovascular Dis- eases, 5, 218-226. http://dx.doi.org/10.4236/wjcd.2015.58025 Arrhythmogenic Ventricular Dysplasia/Cardiomyopathy: Insights from the Rationale of Disease Nomenclature and Clinical Perspectives Aimé Bonny 1,2* , Mohammed A. Talle 3 , Guy Fontaine 4 1 Douala Cardiovascular Research Center, University of Douala, Douala, Cameroon 2 Clinique Paul Picquet, Sens, France 3 Division of Cardiology, Department of Internal Medicine, University of Maiduguri Teaching Hospital/University of Maiduguri, Maiduguri, Nigeria 4 Groupe Hospitalier Pitie Salpetriere, Unite de Rythmologie, Paris, France Email: * aimebonny@yahoo.fr Received 29 May 2015; accepted 23 August 2015; published 26 August 2015 Copyright © 2015 by authors and Scientific Research Publishing Inc. This work is licensed under the Creative Commons Attribution International License (CC BY). http://creativecommons.org/licenses/by/4.0/ Abstract “Arrhythmogenic right ventricular dysplasia” (ARVD), a heart muscle disorder characterized by the presence of fibro-fatty tissue and ventricular electrical vulnerability related to sudden death, was first described in 1977 by a French team. Since then, other terms such as “arrhythmogenic right ventricular cardiomyopathy” (ARVC), “arrhythmogenic cardiomyopathy” (AC), “left-dominant arrhythmogenic cardiomyopathy” (LDAC), and “arrhythmogenic left ventricular dysplasia” (ALVD) have been introduced. These changes in nomenclature of the same disease entity are based on different explanations of pathomorphologic patterns. The dysplasia theory claims cardiac growth “maldevelopment” whereas the cardiomyopathy has been seen as an atrophy from acquired injury (myocyte death) and repair (fibrofatty replacement). The other area of divergent opinion is with regards to involvement of both ventricles rather than being an isolated right ventricular anomaly that may result in increased likelihood of diagnosing the concealed form manifesting with pre- dominant left ventricular arrhythmias. Multiple line of evidences support common disease path- ways: Presence of fibro-fatty and superimposed myocarditis, desmosome mutations and malfunc- tion. These compelling data regarding the heart growth, and pathological, clinical, phenotype/ genotype correlates have advanced our understanding of arrhythmogenic ventricular dysplasia/ cardiomyopathy and increased the diagnostic accuracy as well as providing an avenue for future development of new mechanism-based therapies. * Corresponding author.