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 reproducible ventricular arrhythmias during exercise testing. Typically the onset of ventricular arrhythmias is at around 100-120 bpm. The complexity of these arrhythmias often increases with increasing work load, starting with Ventricular Premature Beats, 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 implantation combined with beta-blockers in CPVT patients who survived a cardiac arrest.[2]
• Avoid competitive and other strenuous exercise

External Links

• GeneReviews CPVT

References

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