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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.

    Related Information

    Reference List

    1. Leenhardt A, Denjoy I, Guicheney P. Catecholaminergic Polymorphic Ventricular Tachycardia. Circulation: Arrhythmia & Electrophysiology. Volume 5, issue 5. October 12, P. 1044-1052.


    2. Roston TM. Implantable cardioverter-defibrillator use in catecholaminergic polymorphic ventricular tachycardia: A systematic review. Heart Rhythm. 2018 Dec;15(12):1791-1799.


    3. Priori SG, Napolitano C, Memmi M, Colombi B, Drago F, Gasparini M, et al. Clinical and molecular characterization of patients with catecholaminergic polymorphic ventricular tachycardia. Circulation. 2002;106(1):69.


    4. Priori SG, Wilde AA, Horie M, Cho Y, Behr ER, Berul C, et al. HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes: document endorsed by HRS, EHRA, and APHRS in May 2013 and by ACCF, AHA, PACES, and AEPC in June 2013. Heart Rhythm. 2013 Dec;10(12):1932-63. Epub 2013 Aug 30.


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    RESOURCE AUTHOR(S)

    Dr Julian Marsden

    Clinical Resources Lead

    Tanis King

    DISCLAIMER

    The purpose of this document is to provide health care professionals with key facts and recommendations for the diagnosis and treatment of patients in the emergency department. This summary was produced by Emergency Care BC (formerly the BC Emergency Medicine Network) and uses the best available knowledge at the time of publication. However, healthcare professionals should continue to use their own judgment and take into consideration context, resources and other relevant factors. Emergency Care BC is not liable for any damages, claims, liabilities, costs or obligations arising from the use of this document including loss or damages arising from any claims made by a third party. Emergency Care BC also assumes no responsibility or liability for changes made to this document without its consent.

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