Cardiac pacing remains one of the most effective means for preventing torsa
de de pointes in patients with long QT syndrome (LQTS), However, fatal arrh
ythmias may occur despite combined therapy with beta blockers and pacing, a
nd it is possible that failure of cardiac pacing for preventing arrhythmias
in the long run is related (at least in part) to suboptimal pacemaker prog
ramming, Preventing sudden pauses may be especially important for preventin
g arrhythmias in the LQTS because such peruses are highly proarrhythmic in
this patient population. Unfortunately, properly functioning pacemakers can
not be expected to prevent postextrasystolic pauses. The use of a pause-pre
vention pacing algorithm-rate smoothing-for preventing pause-dependent tors
ade de pointes is described in 12 patients with cardiac arrest or syncope d
ue to congenital LQTS who were followed for 21 +/- 11 months. (C) 2000 by E
xcerpta Medica, Inc.