[The congenital long QT syndrome and its management with a permanent pacemaker].

Two patients with the congenital form of the long QT syndrome and polymorphous ventricular tachycardias unresponsive to adrenergic beta-blockade, were successfully treated with permanent pacemaker. We discuss the role of cardiac stimulation with pacemakers in the context of all the therapeutic modalities, taking into account the recent knowledge of chromosomal alterations and also in ionic channels.