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Katecholaminerge polymorphe ventrikuläre Tachykardien

Catecholaminergic polymorphic ventricular tachycardia

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An Erratum to this article was published on 08 November 2012

Zusammenfassung

Katecholaminerge polymorphe ventrikuläre Tachykardien (CPVT) sind seltene, angeborene ventrikuläre Rhythmusstörungen, die im Zusammenhang mit adrenerger Aktivierung auftreten und zu Synkopen oder einem plötzlichen Herztod führen können. Sie stellen eine der bedrohlichsten angeborenen kardialen Ionenkanalerkrankungen dar. Mutationen im Ryanodin-Rezeptorgen (RYR2), im Calsequestrin-Gen (CASQ2) sowie im Triadin-Gen (TRDN) wurden als molekulargenetisches Korrelat der CPVT identifiziert. β-Blocker sind Therapie der ersten Wahl, wobei als zusätzliche Therapie Klasse-IC-Antiarrhythmika oder Kalziumantagonisten in Betracht kommen. Bei persistierenden Synkopen trotz medikamentöser Therapie oder nach überlebtem plötzlichem Herztod sollte eine ICD-Implantation erfolgen. Die linkskardiale sympathische Denervation ist im Einzelfall ein ergänzender interventioneller Therapieansatz zur Unterdrückung von CPVT. Alle therapeutischen Maßnahmen sollten von einem strikten Vermeiden von körperlichen Spitzenbelastungen begleitet sein.

Abstract

Catecholaminergic polymorphic ventricular tachycardia (PCVT) is a rare, congenital ventricular tachyarrhythmia which occurs in the setting of adrenergic activation. It potentially leads to syncope and/or sudden cardiac death (SCD). PCVT represents one of the most dangerous congenital ion channel diseases. Mutations of the ryanodine receptor gene (RYR2), the calsequestrin gene (CASQ2), and the triadin gene (TRDN) have been identified as an underlying correlate. β-Blockers are employed as therapy and are sometimes combined with class IC antiarrhythmic drugs, or calcium antagonists of the verapamil type. ICD implantation is recommended in case of persisting syncope in the presence of β-blocker therapy or survived SCD. Left thoracic sympathectomy represents a subsidiary interventional therapy for individual cases. In addition, modifications of the patient’s lifestyle including avoidance of physical stress and heart rates > 120/min are recommended.

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Authors and Affiliations

  1. Abteilung für Rhythmologie Department für Kardiologie und Angiologie, Universitätsklinikum Münster, Albert-Schweitzer Campus 1, 48149, Münster, Deutschland

    G. Frommeyer, C. Pott & L. Eckardt

  2. Institut für Genetik von Herzerkrankungen (IfGH), Department für Kardiologie und Angiologie, Universitätsklinikum Münster, Münster, Deutschland

    E. Schulze-Bahr

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  1. G. Frommeyer
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  2. C. Pott
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  3. L. Eckardt
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  4. E. Schulze-Bahr
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Correspondence to G. Frommeyer.

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Frommeyer, G., Pott, C., Eckardt, L. et al. Katecholaminerge polymorphe ventrikuläre Tachykardien. Herzschr Elektrophys 23, 231–236 (2012). https://doi.org/10.1007/s00399-012-0224-8

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  • DOI: https://doi.org/10.1007/s00399-012-0224-8

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