Abstract: As one of the familial abnormalities of cardiac rhythm, Long QT syndrome (LQTS) has been found in 12 different genes. LQT2 is one of the most common LQTS in China, which can lead to Torsade de Points (TdP) and ventricular fibrillation (VF). Clinically it is characterized by recurrent syncope and sudden cardiac death. The autonomic nervous system plays a vital role in some cases of cardiac arrhythmia through the release of neurotransmitter to regulate ion channel. Recently, studies have shown that vagal nerve can reduce the occurrence of certain kinds of cardiac arrhythmia and may play an important role in maintaining normal cardiac rhythm and rate. This paper mainly reviews the recent advances of LQT2, the complex relationship between vagal nerve and LQT2, and the role of vagal nerve in cardiac arrhythmia.