Abstract
A consistent arrhythmogenic trait in Brugada syndrome cellular phenotype.
Highlights
To cite this version: Zeina Al Sayed, Mariam Jouni, Jean-baptiste Gourraud, Nadjet Belbachir, Julien Barc, et al
Dear Editor, Brugada syndrome (BrS) is an inherited arrhythmic disease predisposing to sudden cardiac death (SCD), characterized by a typical electrocardiogram pattern that includes a J point elevation with a coved type ST segment.[1]
Global cellular electrophysiological phenotype was evaluated with action potential (AP) recordings, but no AP basal parameters segregated BrS hiPSC-CMs, and spontaneous beating frequencies did not differ between all cell lines (Figure S4)
Summary
Dear Editor, Brugada syndrome (BrS) is an inherited arrhythmic disease predisposing to sudden cardiac death (SCD), characterized by a typical electrocardiogram pattern that includes a J point elevation with a coved type ST segment.[1]. We superfused ventricular-like BrS hiPSC-CMs during AP recording with GS-458967 (6-(4(Trifluoromethoxy)phenyl)-3-(trifluoromethyl)-[1,2,4]triazolo[4,3-a]pyridine, which selectively blocks late sodium current),[9] causing full inhibition of INa,L (Figure 3F), and found abolishment of EADs (Figure 3G,3H) and reduced APD90 dispersion (Figure 3I) These data strongly suggested that the abnormal increase of INa,L in BrS hiPSC-CMs is responsible for EADs. Further strengthening the role of the INa,L in the electrical cellular phenotype of BrS, when each ECG parameter was tested for its correlation with either INa,L or INa measured densities, only INa,L density correlated significantly with one sole parameter, i.e., the J point elevation (Table S5).
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