Electrical storm in the setting of Short QT syndrome represents an acute, life-threatening arrhythmic emergency. The abbreviated ventricular refractory period characteristic of this channelopathy can sustain repeated episodes of ventricular fibrillation, demanding urgent, targeted intervention beyond standard resuscitation measures.
Clinical scenario: A patient with known or newly identified Short QT syndrome presenting with recurrent ventricular arrhythmias constituting an electrical storm — multiple episodes in a short period that are not spontaneously terminating or are recurring despite cardioversion.
Electrical storm in Short QT syndrome involves a self-reinforcing cycle of malignant arrhythmias driven by pathologically short repolarisation. General anti-arrhythmic approaches may be insufficient or contraindicated in this distinct genetic substrate, and the choice of pharmacological intervention must account for the underlying channelopathy.
For Short QT syndrome complicated by electrical storm, a specific pharmacological agent — a catecholamine — has been identified in current guidelines as an intervention that may be considered in this scenario. The complete regimen, including selection criteria, contraindications, monitoring parameters, and the broader pharmacological context, is detailed in the full structured protocol.
DOI: 10.1093/eurheartj/ehac262
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