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Catecholaminergic Polymorphic Ventricular Tachycardia

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Management of Cardiac Arrhythmias

Part of the book series: Contemporary Cardiology ((CONCARD))

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Abstract

CPVT is a rare arrhythmogenic disorder characterized by adrenergic-induced bidirectional and polymorphic ventricular tachycardia [VT]. Key features include polymorphic VT reproducibly induced during exercise test, isoproterenol infusion, or emotion and exercise. CPVT occurs in children and adolescents and causes syncope and sudden cardiac death at a young age, in absence of structural heart disease. The resting electrocardiogram (ECG) including the QTc interval is normal. There is a clear correlation between the age of the first syncope and the severity of the disease, with a worst prognosis in case of early occurrence. Mutations in the cardiac ryanodine receptor gene (RyR2) and less frequently in the calsequestrin gene (CASQ2) have been related to the disease. β-Blockers without sympathomimetic activity are clinically effective in reducing syncope. However, arrhythmic event rate on β-blocker therapy remains significant, and combination with pharmacological (flecainide) and non-pharmacological (sympathectomy, ICD) therapies should be discussed. Given the high lethality rate in the absence of treatment, family screening is mandatory.

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Authors and Affiliations

  1. AP-HP, Hôpital Bichat, Département de Cardiologie et Centre de Référence des Maladies Cardiaques Héréditaires, Paris, France

    Antoine Leenhardt & Isabelle Denjoy

  2. Université de Paris INSERM U1166, Paris, France

    Antoine Leenhardt & Isabelle Denjoy

  3. Unité médico-chirurgicale de cardiologie congénitale et pédiatrique, M3C-Necker, Hôpital Universitaire Necker Enfants Malades, Université Paris Descartes, Sorbonne Paris Cité, Paris, France

    Alice Maltret

  4. AMC Heart Center, Department of Clinical and Experimental Cardiology, Academic Medical Center, Amsterdam, the Netherlands

    Krystien V. V. Lieve & Christian van der Werf

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  1. Antoine Leenhardt
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  2. Alice Maltret
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  3. Krystien V. V. Lieve
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  4. Christian van der Werf
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Correspondence to Antoine Leenhardt .

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  1. Lankenau Heart Institute, Thomas Jefferson University, Wynnewood, PA, USA

    Gan-Xin Yan

  2. Lankenau Heart Institute, Thomas Jefferson University, Wynnewood, PA, USA

    Peter R. Kowey

  3. Lankenau Heart Institute, Thomas Jefferson University, Wynnewood, PA, USA

    Charles Antzelevitch

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Leenhardt, A., Maltret, A., Lieve, K.V.V., van der Werf, C., Denjoy, I. (2020). Catecholaminergic Polymorphic Ventricular Tachycardia. In: Yan, GX., Kowey, P., Antzelevitch, C. (eds) Management of Cardiac Arrhythmias. Contemporary Cardiology. Humana, Cham. https://doi.org/10.1007/978-3-030-41967-7_31

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