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呈现 Brugada 综合征心电图特征的致心律失常性右室心肌病的组织病理学特征。

Histopathological characteristics of the arrhythmogenic right ventricular cardiomyopathy presenting the electrocardiographic characteristics with Brugada syndrome.

机构信息

Department of Cardiology, Komaki City Hospital, Komaki, Aichi, Japan.

Department of Cardiovascular Medicine, Nippon Medical School, Tokyo, Japan.

出版信息

J Cardiovasc Electrophysiol. 2023 Sep;34(9):2006-2009. doi: 10.1111/jce.16037. Epub 2023 Aug 9.

DOI:10.1111/jce.16037
PMID:37554112
Abstract

INTRODUCTION

The histopathological characteristics of the overlapping disease states of Brugada syndrome (BrS) and arrhythmogenic right ventricular cardiomyopathy (ARVC) have not been fully elucidated.

METHODS

A 71-year-old man showed coved-type ST-segment elevation with the right precordial leads, and the echocardiography demonstrated right ventricular (RV) dilatation. After 11 months, he died of a polymorphic VT storm.

RESULTS

The pathological tissue demonstrated fibrofatty degeneration in the free wall of the RV outflow tract based on the heart autopsy.

CONCLUSION

The overlapping disease states of BrS and ARVC showed histopathological characteristics consistent with ARVC.

摘要

介绍

Brugada 综合征（BrS）和致心律失常性右心室心肌病（ARVC）重叠疾病状态的组织病理学特征尚未完全阐明。

方法

一名 71 岁男性表现出右胸导联的穹窿型 ST 段抬高，超声心动图显示右心室（RV）扩张。11 个月后，他死于多形性 VT 风暴。

结果

心脏尸检显示 RV 流出道游离壁存在纤维脂肪变性。

结论

BrS 和 ARVC 的重叠疾病状态表现出与 ARVC 一致的组织病理学特征。

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