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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Detalles Bibliográficos
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:
Case Reports
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
Descripción
Sumario: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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