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Abstract
Implantable defibrillators are lifesavers and have improved mortality rates in patients at risk of
sudden death, both in primary and secondary prevention. However, they are unable to modify
the myocardial substrate, which remains susceptible to life-threatening ventricular arrhythmias.
Electrical storm is a clinical entity characterized the recurrence of hemodynamically unstable
ventricular tachycardia and/or ventricular fibrillation, twice or more in 24 hours, requiring
electrical cardioversion or defibrillation. With the arrival of the implantable cardioverterdefibrillator, this definition was broadened, and electrical storm is now defined as the
occurrence of three or more distinct episodes of ventricular tachycardia or ventricular
fibrillation in 24 hours, requiring the intervention of the defibrillator (anti-tachycardia pacing
or shock). Clinical presentation can be very dramatic, with multiple defibrillator shocks and
hemodynamic instability. Managing its acute presentation is a challenge, and mortality is high
both in the acute phase and in the long term. In large clinical trials involving patients implanted
with a defibrillator both for primary and secondary prevention, electrical storm appears to be a
harbinger of cardiac death, with notably high mortality soon after the event. In most cases, the
storm can be interrupted by medical therapy, though transcatheter radiofrequency ablation of
ventricular arrhythmias may be an effective treatment for refractory cases.
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