Abstract
Recurrent, life-threatening ventricular tachyarrhythmias (VTAs) or electrical storm (ES) have been well described in patients with structural heart diseases. However, there are only limited data on ES in patients with J wave syndrome (JWS). ES is an important clinical manifestation of JWS. It can be the first manifestation of JWS, and if not properly managed, may lead to catastrophic outcomes.
When monitored vigilantly, ES is accompanied by dramatic ECG changes. Ventricular fibrillation (VF) episodes are typically initiated by bradycardia or sinus pauses. Prominent bradycardia-dependent augmentation of ER patterns or right precordial J waves is noted before initiation of VF. Traditional antiarrhythmic medications are largely not effective. However, they show dramatic response to intravenous isoproterenol or heart rate elevation. For long-term management of VF episodes, oral quinidine and implantable defibrillators are indicated.
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Nam, GB. (2016). Clinical Features of Electrical Storms Associated with J Wave Syndromes and Acquired Forms of J Wave Syndrome. In: Antzelevitch, C., Yan, GX. (eds) J Wave Syndromes. Springer, Cham. https://doi.org/10.1007/978-3-319-31578-2_14
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