Human Atrial Arrhythmogenesis and Sinus Bradycardia in <i>KCNQ1</i>-Linked Short QT Syndrome: Insights From Computational Modelling.

ABSTRACT: Atrial fibrillation (AF) and sinus bradycardia have been reported in patients with short QT syndrome variant 2 (SQT2), which is underlain by gain-of-function mutations in KCNQ1 encoding the α subunit of channels carrying slow delayed rectifier potassium current, I _Ks. However, the mechanism(s) underlying the increased atrial arrhythmogenesis and impaired cardiac pacemaking activity arising from increased I _Ks remain unclear. Possible pharmacological interventions of AF in the SQT2 condition also remain to be elucidated. Using computational modelling, we assessed the functional impact of SQT2 mutations on human sinoatrial node (SAN) pacemaking, atrial repolarisation and arrhythmogenesis, and efficacy of the anti-arrhythmic drug quinidine. Markov ch

SUBMITTER: Whittaker DG 

PROVIDER: S-EPMC6180159 | biostudies-literature | 2018

REPOSITORIES: biostudies-literature