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

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 mexiletin...

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Autores principales: Fujisawa, Taishi, Aizawa, Yoshiyasu, Katsumata, Yoshinori, Kimura, Kensuke, Hashimoto, Kenji, Yamashita, Terumasa, Miyama, Hiroshi, Kimura, Takehiro, Kosaki, Kenjiro, Takatsuki, Seiji, Shimizu, Wataru, Fukuda, Keiichi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7011830/
https://www.ncbi.nlm.nih.gov/pubmed/32071644
http://dx.doi.org/10.1002/joa3.12300
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author Fujisawa, Taishi
Aizawa, Yoshiyasu
Katsumata, Yoshinori
Kimura, Kensuke
Hashimoto, Kenji
Yamashita, Terumasa
Miyama, Hiroshi
Kimura, Takehiro
Kosaki, Kenjiro
Takatsuki, Seiji
Shimizu, Wataru
Fukuda, Keiichi
author_facet Fujisawa, Taishi
Aizawa, Yoshiyasu
Katsumata, Yoshinori
Kimura, Kensuke
Hashimoto, Kenji
Yamashita, Terumasa
Miyama, Hiroshi
Kimura, Takehiro
Kosaki, Kenjiro
Takatsuki, Seiji
Shimizu, Wataru
Fukuda, Keiichi
author_sort Fujisawa, Taishi
collection PubMed
description 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.
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spelling pubmed-70118302020-02-18 Mexiletine shortens the QT interval in a pedigree of KCNH2 related long QT syndrome Fujisawa, Taishi Aizawa, Yoshiyasu Katsumata, Yoshinori Kimura, Kensuke Hashimoto, Kenji Yamashita, Terumasa Miyama, Hiroshi Kimura, Takehiro Kosaki, Kenjiro Takatsuki, Seiji Shimizu, Wataru Fukuda, Keiichi J Arrhythm Case Reports 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. John Wiley and Sons Inc. 2020-01-08 /pmc/articles/PMC7011830/ /pubmed/32071644 http://dx.doi.org/10.1002/joa3.12300 Text en © 2020 The Authors. Journal of Arrhythmia published by John Wiley & Sons Australia, Ltd on behalf of the Japanese Heart Rhythm Society. This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Reports
Fujisawa, Taishi
Aizawa, Yoshiyasu
Katsumata, Yoshinori
Kimura, Kensuke
Hashimoto, Kenji
Yamashita, Terumasa
Miyama, Hiroshi
Kimura, Takehiro
Kosaki, Kenjiro
Takatsuki, Seiji
Shimizu, Wataru
Fukuda, Keiichi
Mexiletine shortens the QT interval in a pedigree of KCNH2 related long QT syndrome
title Mexiletine shortens the QT interval in a pedigree of KCNH2 related long QT syndrome
title_full Mexiletine shortens the QT interval in a pedigree of KCNH2 related long QT syndrome
title_fullStr Mexiletine shortens the QT interval in a pedigree of KCNH2 related long QT syndrome
title_full_unstemmed Mexiletine shortens the QT interval in a pedigree of KCNH2 related long QT syndrome
title_short Mexiletine shortens the QT interval in a pedigree of KCNH2 related long QT syndrome
title_sort mexiletine shortens the qt interval in a pedigree of kcnh2 related long qt syndrome
topic Case Reports
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7011830/
https://www.ncbi.nlm.nih.gov/pubmed/32071644
http://dx.doi.org/10.1002/joa3.12300
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