Induced pluripotent stem cells as a disease model for studying inherited arrhythmias: promises and hurdles

Abstract

Cardiac ion channel mutations can lead to alterations of action potential and/or conduction properties, and consequently, arrhythmias. Although sudden cardiac death is a common manifestation of inherited arrhythmias, many aspects of the underlying mechanisms remain undefined. In addition to their potential for cell-based therapies, patient-specific induced pluripotent stem cells (iPSCs) also offer an expandable source of human cardiomyocytes for disease modeling, high-throughput drug screening and cardiotoxicity testing. Here, we review current efforts of using iPSC to model monogenetic arrhythmic diseases and discuss the associated challenges.