Electrical storm induced by premature ventricular beat with extremely short coupling interval in a patient with long QT syndrome type 3

Akira Sato; Takuya Takahashi; Seiko Kuwata; Satoshi Nakano; Junichi Koizumi; Hirofumi Saiki; Manami Akasaka

doi:10.1016/j.jelectrocard.2024.153833

Electrical storm induced by premature ventricular beat with extremely short coupling interval in a patient with long QT syndrome type 3

J Electrocardiol. 2024 Nov 22:88:153833. doi: 10.1016/j.jelectrocard.2024.153833. Online ahead of print.

Authors

Akira Sato¹, Takuya Takahashi¹, Seiko Kuwata¹, Satoshi Nakano¹, Junichi Koizumi², Hirofumi Saiki³, Manami Akasaka¹

Affiliations

¹ Division of Pediatric Cardiology, Department of Pediatrics, Iwate Medical University, Yahaba, Japan.
² Department of Cardiovascular surgery, Iwate Medical University, Yahaba, Japan.
³ Division of Pediatric Cardiology, Department of Pediatrics, Iwate Medical University, Yahaba, Japan. Electronic address: hirosaiki-circ@umin.ac.jp.

PMID: 39602950
DOI: 10.1016/j.jelectrocard.2024.153833

Abstract

Here we report the case of a 10-year-old boy with long QT syndrome type 3 (LQT3) who developed refractory torsade de pointes (TdP) associated with ventricular arrhythmia with a short coupling time (VASCT). After implantable cardioverter-defibrillator replacement, an electrical storm occurred, which was irresponsive to the ventricular pacing as high as 120 bpm. Close inspection of the intracardiac potential revealed TdP associated with VASCT. Dexmedetomidine and verapamil were effective in controlling TdP, which allowed management with slower ventricular pace. Our case highlights the importance of focusing on ventricular arrhythmias particularly those with short coupling interval, in LQT3 with refractory TdP. (99 words).

Keywords: Dexmedetomidine; LQT3; Purkinje; Short coupling; Verapamil.