Arrhythmogenic right ventricular dysplasia

被引:146
作者
Fontaine, G
Fontaliran, F
Hébert, JL
Chemla, D
Zenati, O
Lecarpentier, Y
Frank, R
机构
[1] Hop Jean Rostand, Serv Cardiol, F-94200 Ivry, France
[2] Hop Bicetre, Serv Physiol, F-94270 Le Kremlin Bicetre, France
[3] Loa Ensta Ecole Polytech, INSERM U451, F-9112 Palaiseau, France
来源
ANNUAL REVIEW OF MEDICINE | 1999年 / 50卷
关键词
cardiomyopathy; ventricular tachycardia; sudden death;
D O I
10.1146/annurev.med.50.1.17
中图分类号
R-3 [医学研究方法]; R3 [基础医学];
学科分类号
1001 ;
摘要
Arrhythmogenic right ventricular dysplasia (ARVD) is a new form of cardiomyopathy probably more frequent than commonly reported. It is a rare but important cause of sudden arrhythmic death in young, otherwise healthy persons, as well as a subtle cause of congestive heart failure. It may lead to temporary incapacitation with catastrophic consequences. Proper electrocardiographic criteria, echocardiography, nuclear medicine, or magnetic resonance imaging could identify most of these individuals. With the exception of full-thickness histological examination of the right ventricular free wall, contrast ventriculography remains the most definitive standard for a positive diagnosis. The wide clinical spectrum of arrhythmogenic right ventricular cardiomyopathies/dysplasia appears to be the result of one or possibly two factors: (a) replacement of most of the right ventricular myocardium by fat and (b) genetic susceptibility to environmental agents (myocarditis). Current treatment modalities include drug therapy, catheter or surgical ablative techniques, and modern treatments of congestive heart failure. Heart transplant is exceptional. Implantable defibrillators, used alone or in combination with drug therapy, will probably play an increasing role in ARVD and related cardiomyopathies.
引用
收藏
页码:17 / 35
页数:19
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