- 作者：Mark J. Perrin ; Paul Angaran ; Zachary Laksman ; Hanfei Zhang ; Liane F. Porepa ; Julie Rutberg ; Cynthia James ; Andrew D. Krahn ; Daniel P. Judge ; Hugh Calkins ; Michael H. Gollob
- 关键词：arrhythmia ; ARVC ; exercise ; genetics
- 刊名：Journal of the American College of Cardiology
- 出版年：5 November, 2013
- 年：2013
- 卷：62
- 期：19
- 页码：1772-1779
- 全文大小：836 K
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聽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聽= 0.048), premature ventricular contractions in 17 of 30 (57%) compared with 3 of 30 (10%) (p聽= 0.0003), and new QRS terminal activation duration聽鈮?5 ms in 7 of 22 (32%) compared with 2 of 29 (7%) (p聽= 0.03). Superior axis premature ventricular contractions occurred only in gene carriers. In the second phase of the study, the frequency of聽these abnormalities was found to be high in patients with symptomatic ARVC: new epsilon waves appeared in 3聽of聽18聽(17%), superior axis premature ventricular contractions in 21 of 25 (84%), and new terminal activation duration聽鈮?5 ms in 8 of 12 (67%).
Conclusions
Exercise testing exposes a latent electrical substrate in asymptomatic ARVC gene carriers that is shared by patients聽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.