Mexiletine shortens the QT interval in a pedigree of KCNH2 related long QT syndrome

Taishi Fujisawa, Yoshiyasu Aizawa, Yoshinori Katsumata, Kensuke Kimura, Kenji Hashimoto, Terumasa Yamashita, Hiroshi Miyama, Takehiro Kimura, Kenjiro Kosaki, Seiji Takatsuki, Wataru Shimizu, Keiichi Fukuda

Research output: Contribution to journalArticlepeer-review

2 Citations (Scopus)


A 23-year-old female had been suffering from recurrent syncopal episodes during sleep since her childhood. She had a family history of sudden death and her QTc interval was remarkably prolonged to 537 ms A Holter ECG revealed torsade de pointes, corresponding to syncope. She was started on mexiletine and her QTc interval shortened. Her symptoms were controlled after β-blockers and Ca-blockers were added. A genetic analysis with a next generation sequencer identified a frameshift mutation at the C terminus of the KCNH2 gene. Here we present a type 2 long QT syndrome case in which mexiletine was effective.

Original languageEnglish
Pages (from-to)193-196
Number of pages4
Journaljournal of arrhythmia
Issue number1
Publication statusPublished - 2020 Feb 1


  • KCNH2
  • long QT syndrome
  • mexiletine
  • sudden death
  • torsade de pointes

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine


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