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KMID : 1011920130140040014
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International Journal of Arrhythmia
2013 Volume.14 No. 4 p.14 ~ p.19
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Arrhythmogenic right ventricular cardiomyopathy/dysplasia
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Cho Yong-Keun
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Abstract
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Arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) is a type of cardiomyopathy characterized by progressive fibrofatty replacement of the myocardium, ventricular arrhythmia, sudden death, and progressive heart failure. ARVC/D may be an important cause of syncope, ventricular arrhythmias, electrocardiogram abnormalities, and/or nonischemic wall motion abnormalities. However, some patients do not have a typical clinical presentation. Therefore, a high clinical suspicion and extensive studies may be needed to establish the diagnosis of ARVC/D.
Recent progress in diagnostic modalities such as genetic testing and magnetic resonance imaging and a better understanding of the clinical manifestations of ARVC/D may lead to optimal patient management. The primary goal of management is prevention of sudden death. Restriction of intense exercise, pharmacologic treatment, catheter ablation to suppress ventricular arrhythmia, and use of an implantable cardioverter defibrillator are important factors in the treatment of patients with ARVC/D.
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KEYWORD
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arrhythmia , cardiomyopathy , right ventricle
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