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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...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Frontiers Media S.A.
2022
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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. |
format | Online Article Text |
id | pubmed-9453449 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
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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