Successful radiofrequency catheter ablation of a premature ventricular contraction triggering ventricular fibrillation in a patient with short QT syndrome

Key Teaching Points•Short QT syndrome (SQTS) is a rare inherited arrhythmic disease and a cause of sudden cardiac death (SCD). Implantable cardioverter-defibrillator (ICD) can prevent SCD in SQTS.•Quinidine can reduce ICD therapy for ventricular fibrillation (VF) in SQTS. If quinidine fails to reduce multiple ICD therapy, more advanced therapeutic option should be considered.•Recording of premature ventricular complexes (PVCs) that trigger VF in 12-lead electrocardiography will result in a success of radiofrequency catheter ablation to control VF occurrences in SQTS.•The mechanism of PVCs triggering VF has not been evaluated in SQTS. Late-phase 3 early afterdepolarizations might be the most likely mechanism of PVCs in SQTS. •Short QT syndrome (SQTS) is a rare inherited arrhythmic disease and a cause of sudden cardiac death (SCD). Implantable cardioverter-defibrillator (ICD) can prevent SCD in SQTS.•Quinidine can reduce ICD therapy for ventricular fibrillation (VF) in SQTS. If quinidine fails to reduce multiple ICD therapy, more advanced therapeutic option should be considered.•Recording of premature ventricular complexes (PVCs) that trigger VF in 12-lead electrocardiography will result in a success of radiofrequency catheter ablation to control VF occurrences in SQTS.•The mechanism of PVCs triggering VF has not been evaluated in SQTS. Late-phase 3 early afterdepolarizations might be the most likely mechanism of PVCs in SQTS.