Investigation of ventricular arrhythmia mechanisms in hypertrophic cardiomyopathy in mice and men

Sudden cardiac death (SCD), one of the major causes of death in hypertrophic cardiomyopathy (HCM), can occur in otherwise asymptomatic HCM patients. Literature data from HCM mouse models indicate pro-arrhythmic cardiac electrophysiology abnormalities which already occur in pre-hypertrophic disease stages. In the present work, we investigated whether similar abnormalities occur in our Mybpc3-targeted HCM mouse model, human HCM patient samples and induced pluripotent stem cell (hiPSC) models.