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BJA Advance Access originally published online on May 23, 2006
British Journal of Anaesthesia 2006 97(2):150-153; doi:10.1093/bja/ael118
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© The Board of Management and Trustees of the British Journal of Anaesthesia 2006. All rights reserved. For Permissions, please e-mail: journals.permissions@oxfordjournals.org

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. Hinterseer1,*, M. Irlbeck2, L. Ney2, B.-M. Beckmann1, A. Pfeufer3, G. Steinbeck1 and S. Kaab1

1 Department of Medicine I, Klinikum Großhadern LMU München, Germany
2 Department of Anaesthesiology, Klinikum Großhadern LMU München, Germany
3 Institute of Human Genetics, Technical University Munich Germany

*Corresponding author: Department of Medicine I, Klinikum Grosshadern, LMU Munich, 81377 Munich, Germany. E-mail: martin.hinterseer{at}med.uni-muenchen.de

We 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 IKr. 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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