Heart 2000;83:661-666 ( June )

Cardiovascular medicine

Arrhythmias in the congenital long QT syndrome: how often is torsade de pointes pause dependent? S Viskin^a, R Fish^a, D Zeltser^a, B Belhassen^a, K Heller^a, D Brosh^a, S Laniado^a, H V Barron^b

^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

OBJECTIVETo 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.


Keywords: long QT; congenital long QT syndrome; torsade de pointes; ventricular tachycardia

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© 2000 by Heart
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