Referenced in: none

Automatically associated channels: Kv11.1

Title: 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.

Authors: M Hinterseer, M Irlbeck, L Ney, B-M Beckmann, A Pfeufer, G Steinbeck, S Kääb

Journal, date & volume: , 2006 Aug , 97, 150-3

PubMed link: http://www.ncbi.nlm.nih.gov/pubmed/16720674

Abstract
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 I(Kr). This case report illustrates the multivariate nature of long-QT syndrome, and emphasizes the usefulness of a pharmacological test for repolarization abnormalities.