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Synkopen und Ionenkanalerkrankungen

Syncopes and channelopathies

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Herzschrittmachertherapie + Elektrophysiologie Aims and scope Submit manuscript

Zusammenfassung

Synkopen können die Erstmanifestation eines Brugada-Syndroms, Long-QT-Syndroms, Short-QT-Syndroms sowie einer katecholaminergen polymorphen Kammertachykardie (CPVT) sein. Diese seltenen Ionenkanalerkrankungen gehen mit einem erhöhten Risiko für den plötzlichen Herztod durch lebensgefährliche Tachyarrhythmien einher und bedürfen einer kardiologischen Anbindung und spezifischen Therapie. Aufgrund der hohen Prävalenz von Synkopen in der Allgemeinbevölkerung machen diese Kanalopathien nur einen Bruchteil der Synkopenursachen aus. Dennoch sollte an die Diagnose gedacht werden, insbesondere bei jungen, strukturell herzgesunden Patienten mit ungeklärter Synkopenursache, bei typisch-rhythmogener Synkopenanamnese, bei charakteristischen Auffälligkeiten im EKG sowie bei positiver Familienanamnese für Ionenkanalerkrankungen oder den plötzlichen Herztod. Auf der anderen Seite haben Synkopen eine große Bedeutung in der Risikostratifikation bei Patienten mit bereits bekannter Ionenkanalerkrankung. Da sie Ausdruck lebensgefährlicher Tachyarrhythmien sein können, zeigen Synkopen unter Umständen ein erhöhtes individuelles Risiko eines Patienten für den plötzlichen Herztod an. Der prädiktive Wert einer positiven Synkopenanamnese und mögliche therapeutische Konsequenzen unterscheiden sich bei den verschiedenen Ionenkanalerkrankungen. Aufgrund der Seltenheit der Erkrankungen sind die Erfahrungen und Evidenz diesbezüglich jedoch limitiert. Dieser Artikel bietet einen Überblick über typische Befunde, die im Rahmen der Synkopendiagnostik hinweisend auf eine Ionenkanalerkrankung sein können und zeigt den Stellenwert der Synkope in der Risikostratifikation bei Patienten mit bereits diagnostizierter Ionenkanalerkrankung auf.

Abstract

Syncope can be the first manifestation of cardiac channelopathies, namely Brugada syndrome, long QT syndrome, short QT syndrome and catecholaminergic polymorphic ventricular tachycardia (CPVT). Patients affected by these rare diseases are at increased risk for sudden cardiac death due to ventricular tachyarrhythmias and require specific therapy and follow-up. As syncope is common in the general population, only few cases are caused by an underlying channelopathy. Nevertheless, the diagnosis should be considered in young patients with structurally normal hearts, especially if the history of syncope is typical for an arrhythmogenic cause, in the presence of characteristic echocardiogram (ECG) patterns, and if there is a family history of channelopathies or sudden cardiac death. On the other hand, syncope plays an important role in the management of patients with diagnosed channelopathies, as they may indicate an increased risk for sudden cardiac death. The predictive value and consequences for treatment vary between the different channelopathies. However, data on this issue are scarce due to the low prevalence of these diseases. This review highlights typical findings in the medical history and diagnostic tests that may point towards an underlying channelopathy in patients with syncope. It also discusses the prognostic and therapeutic implications of a history of syncope in patients with known channelopathies.

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Authors and Affiliations

  1. Rhythmologie und Elektrophysiologie, Klinik für Kardiologie und Angiologie, Medizinische Hochschule Hannover, Carl-Neuberg-Str. 1, 30625, Hannover, Deutschland

    Johanna Müller-Leisse, Christos Zormpas, Thorben König, David Duncker &  Christian Veltmann

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  1. Johanna Müller-Leisse
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  2. Christos Zormpas
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  3. Thorben König
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Correspondence to Christian Veltmann.

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J. Müller-Leisse, C. Zormpas, T. König, D. Duncker und C. Veltmann geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

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Müller-Leisse, J., Zormpas, C., König, T. et al. Synkopen und Ionenkanalerkrankungen. Herzschr Elektrophys 29, 171–177 (2018). https://doi.org/10.1007/s00399-018-0566-y

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  • DOI: https://doi.org/10.1007/s00399-018-0566-y

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