In silico investigation of a KCNQ1 mutation associated with short QT syndrome.

ABSTRACT: Short QT syndrome (SQTS) is a rare condition characterized by abnormally 'short' QT intervals on the ECG and increased susceptibility to cardiac arrhythmias and sudden death. This simulation study investigated arrhythmia dynamics in multi-scale human ventricle models associated with the SQT2-related V307L KCNQ1 'gain-of-function' mutation, which increases slow-delayed rectifier potassium current (I_Ks). A Markov chain (MC) model recapitulating wild type (WT) and V307L mutant I_Ks kinetics was incorporated into a model of the human ventricular action potential (AP) for investigation of QT interval changes and arrhythmia substrates. In addition, the degree of simulated I_Ks inhibition necessary to normalize the QT interval and terminate re-entry in SQT2 conditio

SUBMITTER: Adeniran I 

PROVIDER: S-EPMC5559555 | biostudies-literature | 2017 Aug

REPOSITORIES: biostudies-literature