Susceptibility to Ventricular Arrhythmias Resulting from Mutations in FKBP1B, PXDNL, and SCN9A Evaluated in hiPSC Cardiomyocytes
(a) ECG analysis of patient MMRL 1126 showing early repolarization phenotype. A prominent ST segment elevation and slurring of the QRS complex is apparent in several leads. (b) Infusion of dobutamine caused the appearance of monomorphic VT in MMRL1126 which persisted after dobutamine was discontinued. (c) The arrhythmogenic focus was ablated and patient subsequently exhibited RBBB apparent in lead V1. (d) Patient was challenged with ajmaline resulting in unmasking of BrS.