This protocol covers Catecholaminergic polymorphic ventricular tachycardia (CPVT) in patients who have experienced arrhythmic syncope or have ECG/Holter-documented bidirectional or polymorphic ventricular tachycardia — but have not suffered an aborted cardiac arrest.
The patient presents with confirmed or clinically suspected CPVT and has either experienced arrhythmic syncope or has documented bidirectional or polymorphic ventricular tachycardia on ECG or Holter monitoring, occurring in the absence of prior aborted cardiac arrest. Reducing adrenergic arrhythmic burden is central to management in this setting.
First-line management combines structured lifestyle modifications — aimed at limiting adrenergic triggers — with pharmacological therapy using a non-selective beta-blocker. The complete protocol specifies agent selection, patient-specific considerations, and the management algorithm.
DOI: 10.1093/eurheartj/ehac262
Avoidance of competitive sports, strenuous exercise, and exposure to stressful environments is recommended in all patients with CPVT.
Beta-blockers, ideally non-selective (nadolol or propranolol) are recommended in all patients with a clinical diagnosis of CPVT.
Exercise restriction and beta-blockers without intrinsic sympathomimetic activity are the first-line therapy for CPVT patients.
Non-selective beta-blockers such as nadolol and propranolol are preferred.
Flecainide should be considered in patients with CPVT who experience recurrent syncope, polymorphic/bidirectional VT, or persistent exertional PVCs, while on beta-blockers at the highest tolerated dose.
ICD implantation should be considered in patients with CPVT who experience arrhythmogenic syncope and/or documented bidirectional/PVT while on highest tolerated beta-blocker dose and on flecainide.
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