Noninvasive Multimodality Imaging in ARVD/C

JACC-CARDIOVASCULAR IMAGING

卷:8

Article

te Riele, Anneline S. J. M.^1,2  Tandri, Harikrishna¹  Sanborn, Danita M.³  Bluemke, David A.^4,5 

[1] Johns Hopkins Univ, Sch Med, Dept Med, Div Cardiol, Baltimore, MD 21205 USA

[2] Univ Med Ctr Utrecht, Dept Med, Div Cardiol, Utrecht, Netherlands

[3] Harvard Univ, Sch Med, Massachusetts Gen Hosp, Dept Med,Div Cardiol, Boston, MA USA

[4] Johns Hopkins Univ, Sch Med, Dept Radiol, Baltimore, MD 21205 USA

[5] NIH, Radiol & Imaging Sci, Ctr Clin, Bethesda, MD 20892 USA

关键词: arrhythmogenic right ventricular dysplasia/cardiomyopathy;    cardiac magnetic resonance;    computed tomography;    echocardiography;    imaging;   

DOI  :  10.1016/j.jcmg.2015.02.007

来源: Elsevier

【 摘 要 】

Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a familial cardiomyopathy resulting in progressive right ventricular (RV) dysfunction and malignant ventricular arrhythmias. Although ARVD/C is generally considered an inherited cardiomyopathy, the arrhythmogenic nature of the disease is striking. Affected individuals typically present in the second to fourth decade of Life with arrhythmias originating from the right ventricle. Over the past decade, pathogenic ARVD/C-causing mutations have been identified in 5 genes encoding the cardiac desmosome. Disruption of the desmosomal connection system between cardiomyocytes may be represented structurally by ventricular enlargement, global or regional contraction abnormalities; RV-aneurysms, or fibrofatty replacement. These abnormalities are typically observed in predilection areas, including the subtricuspid region, basal RV free wall, and Left ventricular posterolateral wall. As such, structural and functional abnormalities on cardiac imaging constitute an important diagnostic criterion for the disease. This paper discusses the current status and role of echocardiography, cardiac magnetic resonance imaging, and computed tomography for suspected ARVD/C. (C) 2015 by the American College of Cardiology Foundation.

【 授权许可】

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