Antiarrhythmic effects of vernakalant in human-induced pluripotent stem cell-derived cardiomyocytes from a patient with short QT syndrome type 1

(1) Background: Short QT syndrome (SQTS) may result in sudden cardiac death. So far, no drugs, except quinidine, have been demonstrated to be effective in some patients with SQTS type 1 (SQTS1). This study was designed to examine the potential effectiveness of vernakalant for treating SQTS1 patients...

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Hauptverfasser: Xu, Qiang (VerfasserIn) , Huang, Xuemei (VerfasserIn) , Meng, Zenghui (VerfasserIn) , Li, Yingrui (VerfasserIn) , Zhong, Rujia (VerfasserIn) , Li, Xin (VerfasserIn) , Cyganek, Lukas (VerfasserIn) , El-Battrawy, Ibrahim (VerfasserIn) , Akın, Ibrahim (VerfasserIn) , Zhou, Xiao-Bo (VerfasserIn) , Lan, Huan (VerfasserIn)
Dokumenttyp: Article (Journal)
Sprache:Englisch
Veröffentlicht: 9 April 2022
In: Journal of cardiovascular development and disease
Year: 2022, Jahrgang: 9, Heft: 4, Pages: 1-14
ISSN:2308-3425
DOI:10.3390/jcdd9040112
Online-Zugang:Verlag, kostenfrei, Volltext: https://doi.org/10.3390/jcdd9040112
Verlag, kostenfrei, Volltext: https://www.mdpi.com/2308-3425/9/4/112
Volltext
Verfasserangaben:Qiang Xu, Xuemei Huang, Zenghui Meng, Yingrui Li, Rujia Zhong, Xin Li, Lukas Cyganek, Ibrahim El-Battrawy, Ibrahim Akin, Xiaobo Zhou and Huan Lan

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246 1 |i Titel des übergeordneten Special issues  |a Takotsubo syndrome, Short QT syndrome and Brugada syndrome 
246 3 3 |a Antiarrhythmic effects of vernakalant in human-induced pluripotent stem cell-derived cardiomyocytes from a patient with short QT syndrome type one 
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520 |a (1) Background: Short QT syndrome (SQTS) may result in sudden cardiac death. So far, no drugs, except quinidine, have been demonstrated to be effective in some patients with SQTS type 1 (SQTS1). This study was designed to examine the potential effectiveness of vernakalant for treating SQTS1 patients, using human-induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs) from a patient with SQTS1. (2) Methods: Patch clamp and calcium imaging techniques were used to examine the drug effects. (3) Results: Vernakalant prolonged the action potential duration (APD) in hiPSC-CMs from a SQTS1-patient (SQTS1-hiPSC-CMs). In spontaneously beating SQTS1-hiPSC-CMs, vernakalant reduced the arrhythmia-like events induced by carbachol plus epinephrine. Vernakalant failed to suppress the hERG channel currents but reduced the outward small-conductance calcium-activated potassium channel current. In addition, it enhanced Na/Ca exchanger currents and late sodium currents, in agreement with its APD-prolonging effect. (4) Conclusions: The results demonstrated that vernakalant can prolong APD and reduce arrhythmia-like events in SQTS1-hiPSC-CMs and may be a candidate drug for treating arrhythmias in SQTS1-patients. 
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