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Case Reports
. 2023 Feb 18;9(5):324-327.
doi: 10.1016/j.hrcr.2023.02.011. eCollection 2023 May.

Circumstance-dependent functional variants in the major long QT syndrome genes in patients with recurrent polymorphic ventricular arrhythmias: A case series

Bram M A van Bakel  1   2 , Thijs P Kerstens  1   2 , Ieva Arts  3 , Reinder Evertz  1 , Rypko Beukema  1 , Mohamed Boulaksil  3
Affiliations
Case Reports

Circumstance-dependent functional variants in the major long QT syndrome genes in patients with recurrent polymorphic ventricular arrhythmias: A case series

Bram M A van Bakel et al. HeartRhythm Case Rep. .
No abstract available

Keywords: Arrhythmia; Genetics; KCNQ1; Long QT syndrome; SCN5A; Torsades de pointes.

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Figures

Figure 1
Figure 1
A: Case 1. Electrocardiogram (ECG) on admission shows sinus rhythm of 75 bpm with a prolonged QTc interval (580 ms, Bazett formula) and multiple polymorphic premature ventricular complexes. B: ECG during admission shows nonsustained polymorphic ventricular tachycardia, junctional escape rhythm, and premature ventricular complexes in bigeminy.
Figure 2
Figure 2
Case 2. Telemetric monitoring at initiation of torsades de pointes. QT-interval duration was 690 ms. Single arrowheads indicate conducted QRS complexes. Double arrowheads indicate premature ventricular complexes. The second premature QRS complex occurs in the relative refractory period (during the T wave), initiating a torsades de pointes polymorphic ventricular arrhythmia. The first premature QRS complex might already be a fusion complex between a sinus conducted complex and a premature ventricular complex, contributing to ventricular dispersion in refractoriness.
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