Purkinje System Hyperexcitability and Ventricular Arrhythmia Risk in Type 3 Long QT Syndrome

Gain-of-function variants in the SCN5A-encoded Nav1.5 sodium channel cause type 3 long QT syndrome (LQT3) and multifocal ectopic Purkinje-related premature contractions (MEPPC). Although the Purkinje system is uniquely sensitive to the action potential prolonging effects of LQT3-causative variants, the existence of additional Purkinje phenotype(s) in LQT3 is unknown.

Source: Heart Rhythm - May 21, 2020 Category: Cardiology Authors: Walid Barake, John R. Giudicessi, Samuel J. Asirvatham, Michael J. Ackerman Source Type: research