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    Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) – Treatment

    Cardiovascular

    Last Updated May 04, 2021
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    By Julian Marsden, Tanis King

    Context

    • Catecholaminergic polymorphic ventricular tachycardia provoked ventricular tachycardia (also called catecholamine provoked ventricular tachycardia/CPVT) is an arrhythmia triggered by physical activity or emotional stress.
    • CPVT typically presents in childhood or adolescence (mean age 7-9 years).
    • May have a family history of juvenile stress-induced syncope.
    • May have a family history of sudden cardiac death.
    • Episodes of ventricular tachycardia can cause dizziness, syncope, and light-headedness.
    • If CPVT is not treated, cardiac arrest and death can occur.
    • Ventricular arrhythmias reproducible on exercise stress testing.
    • Electrocardiographic Features
      • Bidirectional VT
      • Polymorphic VT
      • Ventricular fibrillation
    • Differential diagnosis includes severe digoxin (foxglove) toxicity.

    Recommended Treatment

    Initial Treatment

    • May need cardioversion/defibrillation if unstable but often converts spontaneously.
    • First-line treatment for patients with CPVT is a long-acting, nonselective beta blocker such as nadolol (1-2 mg/kg).
    • Nadolol is the preferred beta blocker because it has a longer duration of action.
    • Propranolol can be used for acute suppression of recurrent polymorphic ventricular tachycardia.

    Long-Term Treatment

    • Placement of implantable cardioverter-defibrillators (ICD) is recommended for patients with sustained ventricular tachycardia, polymorphic ventricular, or ventricular fibrillation in spite of receiving beta blocker therapy.
    • All patients with CPVT should avoid competitive sports and strenuous exercise.
    • Flecainide is recommended for patients taking beta blockers with an ICD who continue to experience ventricular arrhythmias.

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