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Published in: Journal of Cardiovascular Magnetic Resonance 1/2008

Open Access 01-12-2008 | Review

Role of cardiovascular magnetic resonance imaging in arrhythmogenic right ventricular dysplasia

Authors: Aditya Jain, Harikrishna Tandri, Hugh Calkins, David A Bluemke

Published in: Journal of Cardiovascular Magnetic Resonance | Issue 1/2008

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Abstract

Arrhythmogenic right ventricular dysplasia (ARVD) is a genetic cardiomyopathy characterized clinically by ventricular arrhythmias and progressive right ventricular (RV) dysfunction. The histopathologic hallmark is fibro-fatty replacement of RV myocardium. It is inherited in an autosomal pattern with variable penetrance. ARVD is unique in that it most commonly presents in young, otherwise healthy and highly athletic individuals. The cause of ARVD is not well-known but recent evidence suggests strongly that it is a disease of desmosomal dysfunction. The disease involvement is not limited only to the RV as left ventricle (LV) has also been reportedly affected. Diagnosis of ARVD is challenging and is currently based upon a multi-disciplinary work-up of the patient as defined by the Task Force. Currently, implanted cardioverter defibrillators (ICD) are routinely used to prevent sudden death in patients with ARVD. Cardiovascular MR is an important non-invasive diagnostic modality that allows both qualitative and quantitative evaluation of RV. This article reviews the genetics of ARVD, current status and role of CMR in the diagnosis of ARVD and LV involvement in ARVD.
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Metadata
Title
Role of cardiovascular magnetic resonance imaging in arrhythmogenic right ventricular dysplasia
Authors
Aditya Jain
Harikrishna Tandri
Hugh Calkins
David A Bluemke
Publication date
01-12-2008
Publisher
BioMed Central
Published in
Journal of Cardiovascular Magnetic Resonance / Issue 1/2008
Electronic ISSN: 1532-429X
DOI
https://doi.org/10.1186/1532-429X-10-32

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