Arrhythmogenic Right Ventricular Dysplasia

被引：29

作者：

Calkins, Hugh ^{[1
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机构：

[1] Johns Hopkins Univ, Sch Med, Baltimore, MD 21218 USA

[2] Johns Hopkins Univ Hosp, Clin Electrophysiol Lab, Atrial Fibrillat Ctr, Arrhythmia Serv, Baltimore, MD 21287 USA

[3] Johns Hopkins Univ Hosp, Arrhythmogen Right Ventricular Dysplasia Program, Baltimore, MD 21287 USA

[4] Williams Coll, Williamstown, MA 01267 USA

[5] Harvard Univ, Sch Med, Cambridge, MA 02138 USA

[6] Massachusetts Gen Hosp, Boston, MA 02114 USA

[7] Univ Michigan, Ann Arbor, MI 48109 USA

来源：

CURRENT PROBLEMS IN CARDIOLOGY | 2013年 / 38卷 / 03期

关键词：

ELECTROCARDIOGRAPHIC FEATURES; CATHETER ABLATION; WOOLLY HAIR; CARDIOMYOPATHY; DYSPLASIA/CARDIOMYOPATHY; TACHYCARDIA; PLAKOGLOBIN; MUTATIONS; DIAGNOSIS; MURINE;

D O I：

10.1016/j.cpcardiol.2012.12.002

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Arrhythmogenic right ventricular dysplasia (ARVD) is an inherited cardiomyopathy and is also called ARVD/C. In most cases, ARVD is inherited in an autosomal-dominant pattern and clinically is characterized by ventricular arrhythmias with an increased risk of sudden death. Although it is prominently a disease of the right ventricle (RV), involvement of the left ventricle is not uncommon. Pathologically, there is RV myocardial atrophy with thinning of the wall, aneurysm, and global RV dilatation. Genetic testing and magnetic resonance imaging of the RV are an increasing aspect of the diagnosis. Diagnosis relies on a scoring system with major or minor criteria. Prevention of sudden death is the primary goal of management Pharmacologic treatment of arrhythmias, catheter ablation of ventricular tachycardia, and implantable cardioverter defibrillator are the mainstay of treatment of ARVD/C. (Curr Probl Carcliol 2013;38:103-123.)

引用

页码：103 / 123

页数：21

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