10th May 2025

Catecholaminergic Polymorphic Ventricular Tachycardia

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a heritable disorder characterized by catecholamine induced bidirectional VT and polymorphic VT in the absence of structural heart disease or ischaemia.

Clinical feature

• Bidirectional VT or polymorphic VT induced by physical activity or emotional stress
• Most patients have normal ECG and structurally normal heart
• Two main genetic types: dominant disease due to mutation of gene encoding RYR2 receptor and recessive disease due to mutation of gene encoding CASQ2

Investigations

• Exercise stress test: Elicits bidirectional or polymorphic VT (Figure-4)
• Genetic testing 
• Epinephrine or isoprenaline challenge may be considered when exercise testing is not possible

Image Source: Wikimedia Commons (2010). Catecholaminergic polymorphic ventricular tachycardia, Licensed under a creative commons licence. Available for free at:  https://commons.wikimedia.org/wiki/Category:Catecholaminergic_polymorphic_ventricular_tachycardia#/media/File:ECG000033_(CardioNetworks_ECGpedia).jpg (Accessed 7 May 2025).

Management

• Avoidance of competitive sports, strenuous exercise and exposure to stressful environments is recommended to all patients
• Non-selective beta-blockers (nadolol or propranolol) are recommended in all patients.
• ICD implantation is indicated in patients with aborted cardiac arrest in combination with beta-blockers and flecainide 
• Left cardiac sympathetic denervation or Flecainide can be considered when above measures fail.