Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a lethal arrhythmia that is characterized by bidirectional or polymorphic ventricular tachycardia (VT). CPVT leads to ventricular fibrillation (VF) and sudden cardiac death (SCD). Pharmacological therapy, including beta-blockers and flecainide, has been the first-line therapy for CPVT. For medically refractory cases, left cervical sympathetic denervation is another effective treatment option. However, several cases have been described in whom SCD was not prevented because implantable cardioverterdefibrillator (ICD) shock deliveries led to catecholamine release, which resulted in electrical storms.

Although successful catheter ablation for ventricular premature beats (VPBs) triggering VF in CPVT has been reported, the long-term efficacy of catheter ablation of trigger VPBs and complicated atrial arrhythmias has not been determined (1–3). The present study population comprised 5 patients diagnosed with CPVT caused by a mutation of the cardiac ryanodine receptor gene (RYR2). Each study subject had experienced syncope since childhood, occurring at least once every year, up to several times per month.