• Br J Anaesth · Aug 2006

    Case Reports

    Acute respiratory distress syndrome with transiently impaired left ventricular function and Torsades de Pointes arrhythmia unmasking congenital long QT syndrome in a 25-yr-old woman.

    • M Hinterseer, M Irlbeck, L Ney, B-M Beckmann, A Pfeufer, G Steinbeck, and S Kaab.
    • Department of Medicine I, Klinikum Grosshadern, LMU München, Germany. martin.hinterseer@med.uni-muenchen.de
    • Br J Anaesth. 2006 Aug 1; 97 (2): 150-3.

    AbstractWe report a case of recurrent episodes of Torsades de Pointes arrhythmia in the setting of transiently impaired left ventricular ejection fraction, acute respiratory distress syndrome, transient hypokalaemia and QT-prolonging drugs, in a previously healthy 25-yr-old female patient. In the course of the clinical and genetic work-up this patient was newly diagnosed with a mutation in KCNH2 encoding the alpha-subunit of the human repolarizing potassium channel I(Kr). This case report illustrates the multivariate nature of long-QT syndrome, and emphasizes the usefulness of a pharmacological test for repolarization abnormalities.

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