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a Department of
Cardiology, Sourasky-Tel Aviv Medical Center, Sackler-School of
Medicine, Tel Aviv University, Israel, b Cardiovascular Research
Institute, University of California, San Francisco, California, USA
Correspondence to: Dr S Viskin, Department of Cardiology, Tel Aviv Medical Center, Weizman 6, Tel Aviv 64239, Israel email: viskin_s{at}netvision.net.il
Accepted 6 January
2000
OBJECTIVE
To determine the frequency
and predictors of pause dependent torsade de pointes among patients
with the congenital long QT syndrome and spontaneous ventricular tachyarrhythmias.
DESIGNThe literature on the
"congenital long QT" was reviewed. Articles with illustrations
demonstrating the onset of spontaneous polymorphic ventricular
arrhythmias in the absence of arrhythmogenic drugs were included.
RESULTSIllustrations of 62 spontaneous episodes of torsade de pointes among patients with
congenital long QT syndrome were found in the literature. The majority
(74%) of documented arrhythmias were "pause dependent"; 82% of
these pauses were longer than the basic cycle length by > 100 ms.
Age and sex correlated with the mode of arrhythmia initiation.
Arrhythmias in infants (
3 years old) were not pause dependent,
while female sex correlated with pause dependent torsade. Using
multivariate analysis, age was the only independent predictor of the
mode of onset of torsade de pointes.
CONCLUSIONAvailable data suggest
that the majority of spontaneous arrhythmias in the congenital long QT
syndrome are pause dependent. Torsade de pointes that is not preceded
by pauses appears to be limited to patient subgroups with severe forms
of the disease, like symptomatic infants. These findings have important
implications regarding the use of cardiac pacing for arrhythmia prevention.
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