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Long-QT-Syndrom

Historie, Genetik, klinische Symptome, Ursachen und Therapie

Long QT syndrome

History, genetics, clinical symptoms, causes and therapy

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Zusammenfassung

Das Long-QT-Syndrom wird durch eine veränderte kardiale Erregungsrückbildung aufgrund funktioneller Ionenkanaldefekte verursacht. Es wird zwischen einer angeborenen Form („congenital“, cLQTS) und einer erworbenen Form der Erkrankung („acquired“, aLQTS) unterschieden. Folge der Erkrankung ist die namensgebende Verlängerung des QT-Intervalls im Elektrokardiogramm, die eine Prädisposition für Herzrhythmusstörungen und kardiale Ereignisse bis zum plötzlichen Herztod darstellt. Dieser Beitrag gibt einen Überblick über Historie, Genetik, klinische Symptome, Ursachen sowie Therapie des cLQTS und des aLQTS auf Basis aktueller wissenschaftlicher Literatur. Mit Kenntnis der pathophysiologischen Besonderheiten des Krankheitsbilds lässt sich die grundlegende anästhesiologische Vorgehensweise bei Patienten mit LQTS im perioperativen Umfeld ableiten.

Abstract

The long QT syndrome is caused by a change in cardiac repolarization due to functional ion channel defects. A differentiation is made between a congenital (cLQTS) and an acquired (aLQTS) form of the disease. The disease results in the name-giving prolongation of the QT interval in the electrocardiogram and represents a predisposition for cardiac arrhythmia and sudden cardiac death. This article summarizes the current knowledge on the history, pathophysiology, clinical symptoms and therapy of cLQTS and aLQTS. This knowledge of pathophysiological features of the symptoms allows the underlying anesthesiological approach for individualized perioperative concepts for patients suffering from LQTS to be derived.

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Danksagung

Die Autoren bedanken sich bei Herrn Prof. Kääb für seine Anmerkungen und bei GE Deutschland für die freundliche Genehmigung zur Verwendung von Abb. 2, 3 und 5.

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Authors and Affiliations

  1. Klinik für Anästhesiologie und Operative Intensivmedizin, Klinikum Augsburg, Augsburg, Deutschland

    T. Krönauer

  2. Klinik für Anästhesiologie, Operative Intensivmedizin und Schmerztherapie, Städtisches Klinikum München GmbH, Klinikum Bogenhausen, Englschalkinger Str. 77, 81925, München, Deutschland

    P. Friederich

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Correspondence to P. Friederich.

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Interessenkonflikt

T. Krönauer und P. Friederich geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

Additional information

Dieser Betrag ergänzt inhaltlich den CME-Beitrag in dieser Ausgabe: Krönauer T, Friederich P (2015) Perioperative Betreuung von Patienten mit Long-QT-Syndrom. DOI 10.1007/s00101-015-0067-x

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Krönauer, T., Friederich, P. Long-QT-Syndrom. Anaesthesist 64, 586–595 (2015). https://doi.org/10.1007/s00101-015-0068-9

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  • DOI: https://doi.org/10.1007/s00101-015-0068-9

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