Catecholaminergic Polymorphic Ventricular Tachycardia

Author(s)	J.S.S.G. de Jong, MD
Moderator	P.G. Postema, MD
Supervisor
some notes about authorship

The ECG of a patient with CPVT in rest is normal

The ECG of the same patient with CPVT during exercise. Asterisks mark polymorphic ventricular beats.

Catecholaminergic Polymorphic Ventricular Tachycardia is a congenital disease that leads to exercise induced ventricular arrhythmias and / or syncope and carries an increased risk of sudden death.

Characteristics of CPVT:

• The mean onset of arrhythmias is 7-9 years
• Absence of structural cardiac abnormalities
• Normal resting ECG
• Syncope during physical activity or emotional stress

Diagnosis

• The diagnosis is based on the patient's clinical history (dizziness or syncope induced by exercise or emotional stress and a family history containing syncope or sudden death in young relatives related to similar triggers) and reproducible ventricular arrhythmias during exercise testing. The complexity of these arrhythmias often increases with increasing work load, starting with Ventricular Premature Beats, and ending with bidirectional ventricular tachycardia to polymorphic ventricular tachycardia.
• Two genes have been linked to CPVT. Both lead to a defect in intracellular calcium metabolism:
  • the hRyR2 gene, coding for the cardiac ryanodine receptor: (OMIM™ link 180902) (50-55 % of patients)
  • the CASQ2 gene, coding for the calsequestrine protein: (OMIM™ link 114251) (1-2 % of patients)

Treatment[1]

• Beta-blockers
• ICD (Internal Cardioverter Defibrillator) implantation combined with beta-blockers in CPVT patients who survived a cardiac arrest or patients with syncope and/or documented sustained ventricular tachycardia despite beta-blocker therapy.[2]
• Surgical left cardiac sympathetic denervation in selected patients whose symptoms and/or ventricular arrhythmias are not controlled by pharmacologic therapy [3][4]
• Avoid competitive and other strenuous exercise

References

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