Exercise Testing in Asymptomatic Gene Carriers Exposes a Latent Electrical Substrate of Arrhythmogenic Right Ventricular Cardiomyopathy

被引:58
|
作者
Perrin, Mark J. [1 ]
Angaran, Paul [2 ]
Laksman, Zachary [2 ]
Zhang, Hanfei [1 ]
Porepa, Liane F. [2 ]
Rutberg, Julie [1 ]
James, Cynthia [3 ]
Krahn, Andrew D. [2 ]
Judge, Daniel P. [3 ]
Calkins, Hugh [3 ]
Gollob, Michael H. [1 ]
机构
[1] Univ Ottawa, Inst Heart, Dept Med, Div Cardiol, Ottawa, ON K1Y 4W7, Canada
[2] Univ Western Ontario, Dept Med, Div Cardiol, London, ON, Canada
[3] Johns Hopkins Univ, Sch Med, Dept Med Cardiol, Baltimore, MD USA
关键词
arrhythmia; ARVC; exercise; genetics; CRITERIA; RECOMMENDATIONS; DYSPLASIA; SOCIETY; DEATH; YOUNG;
D O I
10.1016/j.jacc.2013.04.084
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Objectives The aim of this study was to determine if exercise testing could expose a latent electrical substrate of arrhythmogenic right ventricular cardiomyopathy (ARVC) in asymptomatic gene carriers. Background Management of asymptomatic ARVC gene carriers is challenging because of variable penetrance of disease and the recognition that sudden cardiac death may be the first clinical manifestation. Methods Exercise-induced abnormalities during exercise treadmill testing (ETT) were initially compared in 60 subjects: 30 asymptomatic ARVC gene carriers and 30 healthy controls. In phase 2 of the study, ETT results of 25 patients with ARVC with histories of sustained ventricular arrhythmia or cardiac arrest were evaluated to determine if ETT abnormalities in asymptomatic gene carriers were common to patients with a malignant electrical form of the disease. Results Depolarization abnormalities during ETT were found to develop more frequently in asymptomatic gene carriers compared with healthy controls: epsilon waves appeared in 4 of 28 (14%) compared with 0 of 30 (0%) (p = 0.048), premature ventricular contractions in 17 of 30 (57%) compared with 3 of 30 (10%) (p = 0.0003), and new QRS terminal activation duration >= 55 ms in 7 of 22 (32%) compared with 2 of 29 (7%) (p = 0.03). Superior axis premature ventricular contractions occurred only in gene carriers. In the second phase of the study, the frequency of these abnormalities was found to be high in patients with symptomatic ARVC: new epsilon waves appeared in 3 of 18 (17%), superior axis premature ventricular contractions in 21 of 25 (84%), and new terminal activation duration >= 55 ms in 8 of 12 (67%). Conclusions Exercise testing exposes a latent electrical substrate in asymptomatic ARVC gene carriers that is shared by patients with ARVC with histories of ventricular arrhythmia. ETT may be useful in guiding treatment decisions, exercise prescription, and prioritizing medical surveillance in asymptomatic ARVC gene carriers. (C) 2013 by the American College of Cardiology Foundation
引用
收藏
页码:1772 / 1779
页数:8
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