Short-Coupled Variant of “Torsades de Pointes” and Polymorphic Ventricular Tachycardia

Introduction

“Torsades de pointes” (TdP) ventricular tachycardias carry a high risk of sudden death, even when they occur in individuals with a structurally normal heart, in the absence of myocardial ischemia or prolonged QT interval. Leenhardt et al described a new syndrome with these characteristics in 1994 that showed a difference in which the TdP were triggered by ventricular extrasystoles (VE) with ultra-short coupling interval (< 300 ms).

Although this condition is easily diagnosed by the described characteristics, there is a lack of data on the clinical management of patients during the phase of electrical storm and during long-term clinical outcome. Over the past 20 years, three patients were identified in our institution with this clinical condition, as well as a family member with VE and short coupling interval, asymptomatic and without documented polymorphic ventricular tachycardia (VT).

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