From the Guidelines
Catecholamine (adrenaline-related) Polymorphic Ventricular Tachycardia (CPVT) is triggered by exertion or emotional stress.
Triggers and Treatment
- The condition is characterized by exertion-related polymorphic or bidirectional VT, associated with syncope and sudden cardiac arrest (SCA) 1.
- Beta blockers, specifically those lacking intrinsic sympathomimetic activity, are the first-line treatment for CPVT, with nadolol being the preferred choice due to its efficacy in reducing adverse cardiac events 1.
- Flecainide can be added to beta blockers in patients with recurrent syncope or polymorphic/bidirectional VT, as it has been shown to suppress ventricular ectopy by up to 76% 1.
- Left cardiac sympathetic denervation may be considered in patients with CPVT who experience recurrent syncope or polymorphic/bidirectional VT despite optimal medical therapy 1.
- ICD implantation is recommended for patients with CPVT who are survivors of cardiac arrest or have a history of exercise- or stress-induced syncope despite optimal medical therapy 1.
- Exercise restriction is also an important aspect of CPVT management, as it can help reduce the risk of arrhythmic events 1.
Key Considerations
- Genetic testing may be useful in confirming the diagnosis of CPVT and identifying affected family members 1.
- ICD programming should be optimized to deliver therapy for ventricular fibrillation and minimize inappropriate shocks 1.
- Verapamil may be considered as an alternative treatment option in patients with CPVT who are intolerant to beta blockers or have contraindications to their use 1.
From the Research
Triggers for Catecholamine Polymorphic Ventricular Tachycardia (CPVT)
- CPVT is triggered by adrenergic stress, such as exercise or emotional stress 2, 3, 4, 5, 6
- The condition is characterized by polymorphic ventricular tachycardia (PVT) or bidirectional ventricular tachycardia, which can lead to syncope or sudden cardiac death 2, 3, 4, 5, 6
- The triggers for CPVT are related to the release of catecholamines, such as adrenaline, which can cause an abnormal heart rhythm 2, 3, 4, 5, 6
Treatment for CPVT
- Standard treatment for CPVT relies on beta-blockers, with add-on therapy such as flecainide and cardiac sympathetic denervation as second-line treatments 2, 3, 5, 6
- An implantable cardioverter-defibrillator is indicated for patients who have suffered a cardiac arrest 2, 3
- Other therapeutic interventions that can be used in conjunction with beta-blockers include moderate exercise training, left cardiac sympathetic denervation, and implantable cardioverter-defibrillators 5
- Potential gene therapy has emerged as a potential treatment for CPVT, although human trials have been limited due to the rare nature of the condition and the high cost of funding 2
Genetic Mutations Associated with CPVT
- CPVT is associated with mutations in several genes, including the ryanodine receptor-2 gene (RyR2) and the calsequestrin-2 gene (CASQ2) 2, 3, 4, 5, 6
- Other genetic variants associated with CPVT include CALM2, TRD, KCNJ2, and ANK2 gene mutations 6
- The incidence of an RyR2 anomaly in CPVTs is about 35-79%, whereas anomalies in the CASQ2 gene account for 3-5% CPVTs 6