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

doi:10.1002/joa3.12300

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 journal › Article › peer-review

2 Citations (Scopus)

Abstract

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 language	English
Pages (from-to)	193-196
Number of pages	4
Journal	journal of arrhythmia
Volume	36
Issue number	1
DOIs	https://doi.org/10.1002/joa3.12300
Publication status	Published - 2020 Feb 1

Keywords

KCNH2
long QT syndrome
mexiletine
sudden death
torsade de pointes

ASJC Scopus subject areas

Cardiology and Cardiovascular Medicine

Access to Document

10.1002/joa3.12300

Cite this

@article{3f4294e12ec947a78770d97918014827,

title = "Mexiletine shortens the QT interval in a pedigree of KCNH2 related long QT syndrome",

abstract = "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.",

keywords = "KCNH2, long QT syndrome, mexiletine, sudden death, torsade de pointes",

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

note = "Funding Information: We are grateful to Mr John Martin for editing this article. This work was supported by MEXT KAKENHI grant number 17K09524. Publisher Copyright: {\textcopyright} 2020 The Authors. Journal of Arrhythmia published by John Wiley & Sons Australia, Ltd on behalf of the Japanese Heart Rhythm Society.",

year = "2020",

month = feb,

day = "1",

doi = "10.1002/joa3.12300",

language = "English",

volume = "36",

pages = "193--196",

journal = "journal of arrhythmia",

issn = "1880-4276",

publisher = "Elsevier BV",

number = "1",

}

TY - JOUR

T1 - Mexiletine shortens the QT interval in a pedigree of KCNH2 related long QT syndrome

AU - Fujisawa, Taishi

AU - Aizawa, Yoshiyasu

AU - Katsumata, Yoshinori

AU - Kimura, Kensuke

AU - Hashimoto, Kenji

AU - Yamashita, Terumasa

AU - Miyama, Hiroshi

AU - Kimura, Takehiro

AU - Kosaki, Kenjiro

AU - Takatsuki, Seiji

AU - Shimizu, Wataru

AU - Fukuda, Keiichi

N1 - Funding Information: We are grateful to Mr John Martin for editing this article. This work was supported by MEXT KAKENHI grant number 17K09524. Publisher Copyright: © 2020 The Authors. Journal of Arrhythmia published by John Wiley & Sons Australia, Ltd on behalf of the Japanese Heart Rhythm Society.

PY - 2020/2/1

Y1 - 2020/2/1

N2 - 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.

AB - 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.

KW - KCNH2

KW - long QT syndrome

KW - mexiletine

KW - sudden death

KW - torsade de pointes

UR - http://www.scopus.com/inward/record.url?scp=85077870814&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85077870814&partnerID=8YFLogxK

U2 - 10.1002/joa3.12300

DO - 10.1002/joa3.12300

M3 - Article

AN - SCOPUS:85077870814

SN - 1880-4276

VL - 36

SP - 193

EP - 196

JO - journal of arrhythmia

JF - journal of arrhythmia

IS - 1

ER -

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

Abstract

Keywords

ASJC Scopus subject areas

Access to Document

Other files and links

Fingerprint

Cite this