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一例致心律失常性右室心肌病(ARVC)的典型病例及文献综述。

A classic case of arrhythmogenic right ventricular cardiomyopathy (ARVC) and literature review.

作者信息

Latt Htun, Tun Aung Thein, Roongsritong Chanwit, Smith David

机构信息

Department of Internal Medicine, University of Nevada, Reno, NV, USA.

Department of Cardiology, Good Samaritan Hospital, Dayton, OH, USA.

出版信息

J Community Hosp Intern Med Perspect. 2017 Jun 6;7(2):115-121. doi: 10.1080/20009666.2017.1302703. eCollection 2017 Mar.

DOI:10.1080/20009666.2017.1302703
PMID:28638576
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5473197/
Abstract

Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) is a relatively under-recognized hereditary cardiomyopathy. It is characterized pathologically by fibro-fatty infiltration of right ventricular (RV) myocardium and clinically by consequences of RV electrical instability. Timely intervention with device therapy and pharmacotherapy may help reduce the risk of arrhythmic events or sudden cardiac death. Here, we describe a classic case of a young adult with ARVC and a brief literature review. The patient presented with exertional palpitations and ARVC was suspected after his routine electrocardiogram (EKG) revealed symmetric T wave inversions and possible epsilon waves in right precordial leads. Subsequent work up showed fatty infiltration of RV myocardium on cardiac magnetic resonance imaging and inducible ventricular tachycardia from the right ventricle during electrophysiologic study. Those findings confirmed the diagnosis of ARVC and warranted treatment with implantable cardioverter defibrillator. It is always exciting to encounter rare pathological entities with classic clinical findings, especially when they present as a diagnostic challenge.We were able to provide correct diagnosis and management, thereby preventing the potentially lethal consequences. Therefore, it is important to recognize the possible EKG findings of ARVC and to know when to pursue further investigations and to implement therapies.

摘要

致心律失常性右室心肌病(ARVC)是一种相对未被充分认识的遗传性心肌病。其病理特征为右心室(RV)心肌的纤维脂肪浸润,临床特征为RV电不稳定的后果。及时进行器械治疗和药物治疗可能有助于降低心律失常事件或心源性猝死的风险。在此,我们描述一例年轻成人ARVC的典型病例并进行简要文献综述。患者表现为劳力性心悸,其常规心电图(EKG)显示右胸前导联对称T波倒置及可能的epsilon波后,怀疑患有ARVC。后续检查显示心脏磁共振成像上RV心肌有脂肪浸润,电生理研究时右心室可诱发室性心动过速。这些发现证实了ARVC的诊断,并需用植入式心脏复律除颤器进行治疗。遇到具有典型临床表现的罕见病理实体总是令人兴奋的,尤其是当它们构成诊断挑战时。我们能够提供正确的诊断和管理,从而预防潜在的致命后果。因此,认识ARVC可能的EKG表现并知道何时进行进一步检查及实施治疗很重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4561/5473197/a30baa676dce/zjch_a_1302703_f0005_b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4561/5473197/34d205b2ad65/zjch_a_1302703_f0001_oc.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4561/5473197/a95457013ce0/zjch_a_1302703_f0002_oc.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4561/5473197/0e6b058d0d80/zjch_a_1302703_f0003_oc.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4561/5473197/3c38f69ea51e/zjch_a_1302703_f0004_b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4561/5473197/a30baa676dce/zjch_a_1302703_f0005_b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4561/5473197/34d205b2ad65/zjch_a_1302703_f0001_oc.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4561/5473197/a95457013ce0/zjch_a_1302703_f0002_oc.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4561/5473197/0e6b058d0d80/zjch_a_1302703_f0003_oc.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4561/5473197/3c38f69ea51e/zjch_a_1302703_f0004_b.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4561/5473197/a30baa676dce/zjch_a_1302703_f0005_b.jpg

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