Literature DB >> 23824749

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D).

V Ramesh Iyer1, Alvin J Chin.   

Abstract

Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a progressive genetic cardiomyopathy characterized by progressive fatty and fibrous replacement of ventricular myocardium. The clinical presentation is marked by ventricular arrhythmias, some fatal. The disease has evolved from a primary electrical/electrophysiological disorder (in the 1980s-1990s) to a diagnostic imaging conundrum (in the 2000s) to the current day understanding of a genetic cardiomyopathy caused by defects in cell-cell adhesion proteins or intracellular signaling components. The pathogenesis, clinical presentation, and the genetics of the disease are discussed in this review.
Copyright © 2013 Wiley Periodicals, Inc.

Entities:  

Keywords:  arrhythmogenic right ventricular cardiomyopathy; desmosome; ventricular arrhythmias; ventricular dysplasia

Mesh：

Substances：

Year:  2013        PMID： 23824749     DOI： 10.1002/ajmg.c.31368

Source DB:  PubMed          Journal:  Am J Med Genet C Semin Med Genet        ISSN： 1552-4868            Impact factor:   3.908


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