Variable Expressivity in Long QT Syndrome Type 2 Rabbits Defines a Low Threshold for Therapeutic IKr Restoration
Long QT syndrome type 2 (LQT2) is caused by pathogenic variants in the hERG potassium channel, resulting in QT prolongation, polymorphic ventricular tachycardia (pVT), and sudden cardiac death. We previously developed a transgenic LQT2 rabbit model that recapitulates the human disease and segregates into two phenotypic...