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The Role of Cardiac Magnetic Resonance Imaging in the Evaluation of Arrhythmogenic Right Ventricular Dysplasia

Authors
Journal
Indian Pacing and Electrophysiology Journal
0972-6292
Publisher
Elsevier
Publication Date
Keywords
  • Review Article
Disciplines
  • Biology
  • Medicine

Abstract

www.ipej.org 503 Review Article The Role of Cardiac Magnetic Resonance Imaging in the Evaluation of Arrhythmogenic Right Ventricular Dysplasia Boban Thomas and Nuno Jalles Tavares Caselas MR Center, Lisbon, Portugal. Address for Correspondence: Boban Thomas, Caselas MR Center, Lisbon, Portugal. E-mail: bobantho/at/gmail.com Keywords: magnetic resonance imaging, arrhythmogenic right ventricular dysplasia Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a cardiac muscle disorder characterized pathologically by fibrofatty replacement of the right ventricular (RV) myocardium [1]. In the early stage of the disease, structural changes may be confined to, the so called "triangle of dysplasia" in the RV. Clinical expression ranges from an asymptomatic phenotype, arrhythmias or even ventricular systolic dysfunction (right or left or both). Although the word 'right' has remained prominent in the description of the disease, left ventricular involvement is now acknowledged at a stage earlier than conventionally acknowledged [2]. ARVC/D is now recognized to be a genetic disease affecting the desmosomes that are responsible for cell-to-cell binding with mutations eventually affecting gap junction functioning [2]. Other mutations in non-desmosomal genes have been described as well. The link between ARVC/D and myocarditis is controversial and unclear despite recent reports that indicate a similar clinical presentation [3,4]. The original 1994 International Task Force criteria (ITFC) for the clinical diagnosis of ARVC/D focused primarily on RV disease manifestations and mandated the absence of or only mild LV involvement to prevent confounding with commoner diseases such as ischemic heart disease and dilated cardiomyopathy, that may also have an arrhythmogenic substrate. The 1994 ITFC were derived from clinical experience gained

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