Abstract: The cardiac QT interval prolongation is one of the important risk factors of clinical malignant arrhythmias and sudden cardiac death. The genome-wide association study (GWAS) recently indicates that inherited defects of known genes regulating cardiac repolarization may be the pathogenic mechanisms of acquired QT prolongation. These gene defects are complicated and closely associated with myocardial electrophysiology. This paper overviews recent progresses in QT-related gene research, aims to explore its relationship with cardiac electrophysiological function and provide a new perspective for studying on mechanisms of QT prolongation related arrhythmias.