In vivo effects of mutant HERG K+ channel inhibition by disopyramide in patients with a short QT-1 syndrome: a pilot study.

@article{Schimpf2007InVE,
  title={In vivo effects of mutant HERG K+ channel inhibition by disopyramide in patients with a short QT-1 syndrome: a pilot study.},
  author={Rainer Schimpf and Christian Veltmann and Carla Giustetto and Fiorenzo Gaita and Martin Borggrefe and Christian Wolpert},
  journal={Journal of cardiovascular electrophysiology},
  year={2007},
  volume={18 11},
  pages={1157-60}
}
INTRODUCTION Quinidine has been evaluated in patients with a short QT-1 syndrome caused by an IKr gain-of-function mutation of HERG. Recently, in vitro data with disopyramide showed an even stronger effect on the N588K mutant current. The aim of the present study was to test the in vivo effects of disopyramide in patients with short QT-1 syndrome caused by a N588K mutation in HERG. METHODS AND RESULTS Repetitive ECGs were recorded in two female patients with short QT-1 syndrome with a N588K… CONTINUE READING

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