Two patients with long QT syndrome, who had episodes of syncope, underwent recordings of the monophasic action potential (MAP) from the right ventricle. Intracoronary administration of acetylcholine (ACh) induced prolongation of MAP duration and caused Torsade de Pointes (Tdp) in both patients. In one patient, intravenous atropine administration did not induce any change in MAP duration. In the other patient, ACh was administered after atropine. According to the results of the present study, abnormal regulation of the muscarinic receptor-mediated K-channel may be involved in the mechanism causing prolongation of MAP duration caused by ACh administration.