Different responses to exercise between Andersen-Tawil syndrome and catecholaminergic polymorphic ventricular tachycardia

Yuko Y. Inoue, Takeshi Aiba, Hiro Kawata, Tomoko Sakaguchi, Wataru Mitsuma, Hiroshi Morita, Takashi Noda, Hiroshi Takaki, Keiko Toyohara, Yoshiaki Kanaya, Toshiyuki Itoi, Takeshi Mitsuhashi, Naokata Sumitomo, Yongkeun Cho, Satoshi Yasuda, Shiro Kamakura, Kengo Kusano, Yoshihiro Miyamoto, Minoru Horie, Wataru Shimizu

Research output: Contribution to journalArticle

Abstract

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.

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Andersen Syndrome
Exercise
Bundle-Branch Block
Cardiac Arrhythmias
Ventricular Premature Complexes
Exercise Test
Polymorphic catecholergic ventricular tachycardia
Ryanodine Receptor Calcium Release Channel
Mutation

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Physiology (medical)

Cite this

Different responses to exercise between Andersen-Tawil syndrome and catecholaminergic polymorphic ventricular tachycardia. / Inoue, Yuko Y.; Aiba, Takeshi; Kawata, Hiro; Sakaguchi, Tomoko; Mitsuma, Wataru; Morita, Hiroshi; Noda, Takashi; Takaki, Hiroshi; Toyohara, Keiko; Kanaya, Yoshiaki; Itoi, Toshiyuki; Mitsuhashi, Takeshi; Sumitomo, Naokata; Cho, Yongkeun; Yasuda, Satoshi; Kamakura, Shiro; Kusano, Kengo; Miyamoto, Yoshihiro; Horie, Minoru; Shimizu, Wataru.

In: Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology, Vol. 20, No. 10, 01.10.2018, p. 1675-1682.

Research output: Contribution to journalArticle

Inoue, YY, Aiba, T, Kawata, H, Sakaguchi, T, Mitsuma, W, Morita, H, Noda, T, Takaki, H, Toyohara, K, Kanaya, Y, Itoi, T, Mitsuhashi, T, Sumitomo, N, Cho, Y, Yasuda, S, Kamakura, S, Kusano, K, Miyamoto, Y, Horie, M & Shimizu, W 2018, 'Different responses to exercise between Andersen-Tawil syndrome and catecholaminergic polymorphic ventricular tachycardia', Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology, vol. 20, no. 10, pp. 1675-1682. https://doi.org/10.1093/europace/eux351
Inoue, Yuko Y. ; Aiba, Takeshi ; Kawata, Hiro ; Sakaguchi, Tomoko ; Mitsuma, Wataru ; Morita, Hiroshi ; Noda, Takashi ; Takaki, Hiroshi ; Toyohara, Keiko ; Kanaya, Yoshiaki ; Itoi, Toshiyuki ; Mitsuhashi, Takeshi ; Sumitomo, Naokata ; Cho, Yongkeun ; Yasuda, Satoshi ; Kamakura, Shiro ; Kusano, Kengo ; Miyamoto, Yoshihiro ; Horie, Minoru ; Shimizu, Wataru. / Different responses to exercise between Andersen-Tawil syndrome and catecholaminergic polymorphic ventricular tachycardia. In: Europace : European pacing, arrhythmias, and cardiac electrophysiology : journal of the working groups on cardiac pacing, arrhythmias, and cardiac cellular electrophysiology of the European Society of Cardiology. 2018 ; Vol. 20, No. 10. pp. 1675-1682.
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abstract = "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.",
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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

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.

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