creators_name: Indik, Julia H. creators_name: Marcus, Frank I. editors_name: Singh, Balbir editors_name: Lokhandwala, Yash editors_name: Francis, Johnson editors_name: Gupta, Anup type: journalp datestamp: 2005-04-24 lastmod: 2011-03-11 08:55:59 metadata_visibility: show title: Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia ispublished: pub subjects: ipej full_text_status: public keywords: Arrhythmogenic right ventricular cardiomyopathy abstract: Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is characterized by the patchy replacement of myocardium by fatty or fibrofatty tissue. These changes lead to structural abnormalities including right ventricular enlargement and wall motion abnormalities that can be detected by echocardiography, angiography, and cine MRI. ARVC/D is a genetically heterogeneous disorder, since it has been linked to several chromosomal loci. Myocarditis may also be a contributing etiological factor. Patients are typically diagnosed during adolescence or young adulthood. Presenting symptoms are generally related to ventricular arrhythmias. Concern for the risk of sudden cardiac death may lead to the implantation of an intracardiac defibrillator. An ongoing multicenter international registry should further our understanding of this disease. date: 2003-07 date_type: published publication: Indian Pacing and Electrophysiology Journal volume: 3 number: 3 publisher: Indian Pacing and Electrophysiology Group pagerange: 148-156 refereed: TRUE referencetext: 1. Rampazzo A. Nava A. Malacrida S. Beffagna G. Bauce B. Rossi V. Zimbello R. Simionati B. Basso C. Thiene G. Towbin JA. Danieli GA. Mutation in human desmoplakin domain binding to plakoglobin causes a dominant form of arrhythmogenic right ventricular cardiomyopathy. American Journal of Human Genetics. 2002;71:1200-6 2. McKoy G. Protonotarios N. Crosby A. Tsatsopoulou A. Anastasakis A. Coonar A. Norman M. Baboonian C. Jeffery S. 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[Journal (Paginated)] document_url: http://cogprints.org/4279/1/indik.htm