Recording of monophasic action potentials of the right ventricle in long QT syndromes complicated by severe ventricular arrhythmias.

Monophasic action potentials (MAPs) were recorded with intracardiac suction electrodes in several areas of the right ventricle in 10 patients with long QT syndromes (with "torsades de pointe') of different etiology. In all cases two characteristic electrophysiological features were observed: (1) a marked difference in MAP duration in the different areas of the right ventricle (asynchronous repolarization); (2) an alteration in the shape of the longest MAPs consisting in humps which occurred on the repolarization phase of MAP. Humps may be interpreted as delayed repolarization phenomena probably due to a decrease in potassium conductance of some ventricular cells. These may lead to focal re-excitation as a result of partial membrane depolarization. Focal re-excitation seems to play a key role in the genesis of severe arrhythmias occurring in the above mentioned syndromes.