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普罗帕酮致致心律失常性右室心肌病患儿QRS波增宽:一例报告及文献复习

Propafenone-Induced QRS Widening in a Child With Arrhythmogenic Right Ventricular Cardiomyopathy: A Case Report and Literatures Review.

作者信息

Chu Yan-Qiu, Wang Ce, Li Xue-Mei, Wang Hong

机构信息

Department of Pediatrics, Shengjing Hospital, China Medical University, Shenyang, China.

出版信息

Front Pediatr. 2020 Oct 30;8:481330. doi: 10.3389/fped.2020.481330. eCollection 2020.

DOI:10.3389/fped.2020.481330
PMID:33194879
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7661465/
Abstract

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a rare cardiac disease in children, and can lead to sudden cardiac death (SCD). Propafenone is classI antiarrhythmic medication, and its side effects include cardiovascular compromise in the form of hypotension, bradycardia, ventricular dysrhythmias, QRS widening, and heart block. Propafenone has been reported causing QRS widening, but rarely in children. In this article, we presented a boy diagnosed with ARVC who meets diagnosis criteria based on typical symptoms, electrocardiograph (ECG), echocardiography (Echo), cardiac magnetic resonance imaging (CMRI), sudden death of first family member, and genetic mutation in desmosomal DSG2 gene. Antiarrhythmic drugs have been used for treating patients with ARVC, by eliminating or decreasing the occurring frequency of arrhythmias. As his ECG showed frequent premature ventricular contractions (PVC), he was prescribed with oral propafenone. One day after the drug treatment, he presented dizziness accompanied with significant QRS widening in ECG. His dizziness was improved when Propafenone dose was reduced, and resolved after sotalol replacement, with ECG recovered to nearly normal state of QRS. Propafenone may lead to QRS widening and increase the risk of ventricular tachycardia, and it may not reduce ARVC associated mortality. This report may serve as a precaution for clinicians when providing cares for ARVC patients.

摘要

致心律失常性右室心肌病(ARVC)是一种儿童罕见的心脏疾病,可导致心源性猝死(SCD)。普罗帕酮是Ⅰ类抗心律失常药物,其副作用包括以低血压、心动过缓、室性心律失常、QRS波增宽和心脏传导阻滞形式出现的心血管功能损害。已有报道普罗帕酮可导致QRS波增宽,但在儿童中罕见。在本文中,我们介绍了一名被诊断为ARVC的男孩,他基于典型症状、心电图(ECG)、超声心动图(Echo)、心脏磁共振成像(CMRI)、家族中首例成员猝死以及桥粒蛋白DSG2基因突变符合诊断标准。抗心律失常药物已被用于治疗ARVC患者,以消除或降低心律失常的发生频率。由于他的心电图显示频发室性早搏(PVC),给他开了口服普罗帕酮。药物治疗一天后,他出现头晕,心电图显示QRS波显著增宽。当降低普罗帕酮剂量时,他的头晕症状有所改善,换用索他洛尔后头晕症状消失,心电图恢复到QRS波接近正常的状态。普罗帕酮可能导致QRS波增宽并增加室性心动过速的风险,且可能不会降低ARVC相关的死亡率。本报告可为临床医生在护理ARVC患者时提供警示。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b9cc/7661465/9aed076092c2/fped-08-481330-g0003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b9cc/7661465/4b795bab12ab/fped-08-481330-g0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b9cc/7661465/a4ef8ab7328f/fped-08-481330-g0002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b9cc/7661465/9aed076092c2/fped-08-481330-g0003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b9cc/7661465/4b795bab12ab/fped-08-481330-g0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b9cc/7661465/a4ef8ab7328f/fped-08-481330-g0002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b9cc/7661465/9aed076092c2/fped-08-481330-g0003.jpg

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Hum Genome Var. 2019 Aug 21;6:38. doi: 10.1038/s41439-019-0069-3. eCollection 2019.
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A founder homozygous DSG2 variant in East Asia results in ARVC with full penetrance and heart failure phenotype.在东亚,DSG2 基因的纯合突变会导致完全外显率的 ARVC 和心力衰竭表型。
Int J Cardiol. 2019 Jan 1;274:263-270. doi: 10.1016/j.ijcard.2018.06.105. Epub 2018 Jun 28.
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Case report of familial sudden cardiac death caused by a DSG2 p.F531C mutation as genetic background when carrying with heterozygous KCNE5 p.D92E/E93X mutation.
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携带杂合KCNE5 p.D92E/E93X突变时,以DSG2 p.F531C突变为遗传背景的家族性心脏性猝死病例报告。
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