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Updated: Jul 2, 2026

Determining the Likelihood of Variant Pathogenicity Using Amino Acid-level Signal-to-Noise Analysis of Genetic Variation
Published on: January 16, 2019
Short-coupled variant of torsade de pointes
K Bogaard1, M S van der Steen, H L Tan
1Department of Cardiology, Kennemer Gasthuis, Haarlem, the Netherlands.
Abstract:
A 36-year-old man with a history of primary sclerosing cholangitis and epilepsy was admitted to our hospital for cholangitis. During admission he was resuscitated because of ventricular fibrillation. ECGs showed multiple ventricular premature beats (VPBs) with a short coupling interval (240 ms), resulting in frequent torsade de pointes (TdP). In total, the patient had to be defibrillated 12 times. Short-coupled TdP is a rare variant of polymorphic ventricular tachycardia, with unknown aetiology. Verapamil seems to be the only drug able to suppress the arrhythmia. Verapamil, however, does not lower the risk of sudden death; therefore, an ICD implantation is advised. (Neth Heart J 2008;16:246-9.).
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