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Case report: Mexiletine suppresses ventricular arrhythmias in Andersen-Tawil syndrome

It is arduous to determine clinical solutions for Andersen-Tawil syndrome (ATS) in patients intolerant of β-blocker. Here, we present the case of a 7-year-old boy with periodic paralysis and dysmorphic features who experienced syncope four times during exercise. His ECG revealed enlarged U waves and...

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Autores principales: Yang, Jing, Li, Kun, Lv, Tingting, Xie, Ying, Liu, Fang, Zhang, Ping
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9453449/
https://www.ncbi.nlm.nih.gov/pubmed/36093155
http://dx.doi.org/10.3389/fcvm.2022.992185
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author Yang, Jing
Li, Kun
Lv, Tingting
Xie, Ying
Liu, Fang
Zhang, Ping
author_facet Yang, Jing
Li, Kun
Lv, Tingting
Xie, Ying
Liu, Fang
Zhang, Ping
author_sort Yang, Jing
collection PubMed
description It is arduous to determine clinical solutions for Andersen-Tawil syndrome (ATS) in patients intolerant of β-blocker. Here, we present the case of a 7-year-old boy with periodic paralysis and dysmorphic features who experienced syncope four times during exercise. His ECG revealed enlarged U waves and QU-prolongation associated with ATS-specific U wave patterns, frequent PVCs, and non-sustained bidirectional or polymorphic ventricular tachycardia. The genetic test showed a de novo missense R218W mutation of KCNJ2. With the diagnosis of ATS and intolerance of β-blocker, the patient was prescribed oral medications of mexiletine 450 mg/day without severe adverse effects. The repeat ECG showed decreased PVC burden from 38 to 3% and absence of ventricular tachycardia. He remained symptom-free during over 2 years of outpatient follow-up. This case demonstrates a new anti-arrhythmic therapy with mexiletine for prevention of life-threatening cardiac events in patients with ATS who are intolerant of β-blocker treatment.
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spelling pubmed-94534492022-09-09 Case report: Mexiletine suppresses ventricular arrhythmias in Andersen-Tawil syndrome Yang, Jing Li, Kun Lv, Tingting Xie, Ying Liu, Fang Zhang, Ping Front Cardiovasc Med Cardiovascular Medicine It is arduous to determine clinical solutions for Andersen-Tawil syndrome (ATS) in patients intolerant of β-blocker. Here, we present the case of a 7-year-old boy with periodic paralysis and dysmorphic features who experienced syncope four times during exercise. His ECG revealed enlarged U waves and QU-prolongation associated with ATS-specific U wave patterns, frequent PVCs, and non-sustained bidirectional or polymorphic ventricular tachycardia. The genetic test showed a de novo missense R218W mutation of KCNJ2. With the diagnosis of ATS and intolerance of β-blocker, the patient was prescribed oral medications of mexiletine 450 mg/day without severe adverse effects. The repeat ECG showed decreased PVC burden from 38 to 3% and absence of ventricular tachycardia. He remained symptom-free during over 2 years of outpatient follow-up. This case demonstrates a new anti-arrhythmic therapy with mexiletine for prevention of life-threatening cardiac events in patients with ATS who are intolerant of β-blocker treatment. Frontiers Media S.A. 2022-08-25 /pmc/articles/PMC9453449/ /pubmed/36093155 http://dx.doi.org/10.3389/fcvm.2022.992185 Text en Copyright © 2022 Yang, Li, Lv, Xie, Liu and Zhang. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Cardiovascular Medicine
Yang, Jing
Li, Kun
Lv, Tingting
Xie, Ying
Liu, Fang
Zhang, Ping
Case report: Mexiletine suppresses ventricular arrhythmias in Andersen-Tawil syndrome
title Case report: Mexiletine suppresses ventricular arrhythmias in Andersen-Tawil syndrome
title_full Case report: Mexiletine suppresses ventricular arrhythmias in Andersen-Tawil syndrome
title_fullStr Case report: Mexiletine suppresses ventricular arrhythmias in Andersen-Tawil syndrome
title_full_unstemmed Case report: Mexiletine suppresses ventricular arrhythmias in Andersen-Tawil syndrome
title_short Case report: Mexiletine suppresses ventricular arrhythmias in Andersen-Tawil syndrome
title_sort case report: mexiletine suppresses ventricular arrhythmias in andersen-tawil syndrome
topic Cardiovascular Medicine
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9453449/
https://www.ncbi.nlm.nih.gov/pubmed/36093155
http://dx.doi.org/10.3389/fcvm.2022.992185
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