TY - JOUR
T1 - Different responses to exercise between Andersen–Tawil syndrome and catecholaminergic polymorphic ventricular tachycardia
AU - Inoue, Yuko Y.
AU - Aiba, Takeshi
AU - Kawata, Hiro
AU - Sakaguchi, Tomoko
AU - Mitsuma, Wataru
AU - Morita, Hiroshi
AU - Noda, Takashi
AU - Takaki, Hiroshi
AU - Toyohara, Keiko
AU - Kanaya, Yoshiaki
AU - Itoi, Toshiyuki
AU - Mitsuhashi, Takeshi
AU - Sumitomo, Naokata
AU - Cho, Yongkeun
AU - Yasuda, Satoshi
AU - Kamakura, Shiro
AU - Kusano, Kengo
AU - Miyamoto, Yoshihiro
AU - Horie, Minoru
AU - Shimizu, Wataru
N1 - Funding Information:
We thank Naotaka Ohta, Hiromi Fujiyama, and Toshiko Shibata for their excellent work on molecular screening. T.A., H.M., N.S., S.K., M.H., and W.S. were supported in part by a Research Grant for Cardiovascular Diseases (H24-033, H26-040) from the Ministry of Health, Labor and Welfare, Japan.
Funding Information:
T.A., H.M., N.S., S.K., M.H., and W.S. were supported in part Research Grant for Cardiovascular Diseases (H24-033, H26-040) the Ministry of Health, Labor and Welfare, Japan.
Publisher Copyright:
Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2017.
PY - 2018/10/1
Y1 - 2018/10/1
N2 - Aims Andersen–Tawil Syndrome (ATS) and catecholaminergic polymorphic ventricular tachycardia (CPVT) are both inherited arrhythmic disorders characterized by bidirectional ventricular tachycardia (VT). The aim of this study was to evaluate the diagnostic value of exercise stress tests for differentiating between ATS and CPVT. Methods and results We included 26 ATS patients with KCNJ2 mutations from 22 families and 25 CPVT patients with RyR2 mutations from 22 families. We compared the clinical and electrocardiographic (ECG) characteristics, responses of ventricular arrhythmias (VAs) to exercise testing, and the morphology of VAs between ATS and CPVT patients. Ventricular arrhythmias were more frequently observed at baseline in ATS patients compared with CPVT patients [the ratio of ventricular premature beats (VPBs)/sinus: 0.83 ± 1.87 vs. 0.06 ± 0.30, P = 0.01]. At peak exercise, VAs were suppressed in ATS patients, whereas they were increased in CPVT patients (0.14 ± 0.40 vs. 1.94 ± 2.71, P < 0.001). Twelve-lead ECG showed that all 25 VPBs and 15 (94%) of 16 bidirectional VTs were right bundle branch block (RBBB) morphology in ATS patients, whereas 19 (86%) of 22 VPBs had left bundle branch block (LBBB), and 12 (71%) of 17 bidirectional VT had LBBB and RBBB morphologies in CPVT patients. Conclusion In patients with ATS, VAs with RBBB morphology were frequently observed at baseline and suppressed at peak exercise. In contrast, exercise provoked VAs with mainly LBBB morphology in patients with CPVT. In adjunct to clinical and baseline ECG assessments, exercise testing might be useful for making the diagnosis of ATS vs. CPVT, both characterized by bidirectional VT.
AB - Aims Andersen–Tawil Syndrome (ATS) and catecholaminergic polymorphic ventricular tachycardia (CPVT) are both inherited arrhythmic disorders characterized by bidirectional ventricular tachycardia (VT). The aim of this study was to evaluate the diagnostic value of exercise stress tests for differentiating between ATS and CPVT. Methods and results We included 26 ATS patients with KCNJ2 mutations from 22 families and 25 CPVT patients with RyR2 mutations from 22 families. We compared the clinical and electrocardiographic (ECG) characteristics, responses of ventricular arrhythmias (VAs) to exercise testing, and the morphology of VAs between ATS and CPVT patients. Ventricular arrhythmias were more frequently observed at baseline in ATS patients compared with CPVT patients [the ratio of ventricular premature beats (VPBs)/sinus: 0.83 ± 1.87 vs. 0.06 ± 0.30, P = 0.01]. At peak exercise, VAs were suppressed in ATS patients, whereas they were increased in CPVT patients (0.14 ± 0.40 vs. 1.94 ± 2.71, P < 0.001). Twelve-lead ECG showed that all 25 VPBs and 15 (94%) of 16 bidirectional VTs were right bundle branch block (RBBB) morphology in ATS patients, whereas 19 (86%) of 22 VPBs had left bundle branch block (LBBB), and 12 (71%) of 17 bidirectional VT had LBBB and RBBB morphologies in CPVT patients. Conclusion In patients with ATS, VAs with RBBB morphology were frequently observed at baseline and suppressed at peak exercise. In contrast, exercise provoked VAs with mainly LBBB morphology in patients with CPVT. In adjunct to clinical and baseline ECG assessments, exercise testing might be useful for making the diagnosis of ATS vs. CPVT, both characterized by bidirectional VT.
KW - Andersen–Tawil syndrome
KW - Catecholaminergic polymorphic ventricular tachycardia
KW - Diagnosis
KW - Exercise
KW - Inherited arrhythmic disorders
KW - Tachyarrhythmias
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U2 - 10.1093/europace/eux351
DO - 10.1093/europace/eux351
M3 - Article
C2 - 29309601
AN - SCOPUS:85054890494
VL - 20
SP - 1675
EP - 1682
JO - Europace
JF - Europace
SN - 1099-5129
IS - 10
ER -