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European Heart Journal Advance Access originally published online on February 15, 2007
European Heart Journal 2007 28(5):529-530; doi:10.1093/eurheartj/ehl530
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© The European Society of Cardiology 2007. All rights reserved. For Permissions, please e-mail: journals.permissions@oxfordjournals.org

Arrhythmogenic right ventricular cardiomyopathy: a ‘final common pathway’ that defines clinical phenotype

Matteo Vatta1, Frank Marcus2 and Jeffrey A. Towbin1,*

1 Department of Pediatrics, Section of Pediatric Cardiology, Baylor College of Medicine, Texas Children's Hospital, 6621 Fannin Street, MC 19345-C, Houston, TX 77030, USA
2 Department of Medicine, University of Arizona, Tucson, AZ, USA

* Corresponding author. Tel: +1 832 826 5651; fax: +1 832 825 5921. E-mail address: jtowbin@bcm.tmc.edu

This editorial refers to ‘Desmoglein-2 mutations in arrhythmogenic right ventricular cardiomyopathy: a genotype-phenotype characterization of familial disease’ {dagger} by P. Syrris et al., on page 581

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Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a primary heart muscle disease with distinct characteristics. ARVD/C predominantly affects the right ventricle (RV), with RV dilation and thinning due to fibrofatty infiltration of the ventricular myocardium, and ultimately depressed systolic function leading to right heart failure or biventricular failure.1 Early in its clinical course, ARVD/C typically presents with ventricular arrhythmias (usually with a left bundle branch pattern), syncope, or sudden cardiac death.2 Tragically, this clinical scenario commonly occurs in young, healthy, athletic individuals. A set of clinical criteria, known as the ‘Task Force Criteria’, first described by McKenna et al.3 in 1994 and later modified for inclusion of family members,4 utilizes . . . [Full Text of this Article]


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Related articles in EHJ:

Desmoglein-2 mutations in arrhythmogenic right ventricular cardiomyopathy: a genotype–phenotype characterization of familial disease
Petros Syrris, Deirdre Ward, Angeliki Asimaki, Alison Evans, Srijita Sen-Chowdhry, Sian E. Hughes, and William J. McKenna
EHJ 2007 28: 581-588. [Abstract] [FREE Full Text]