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

Yosuke Murase; Osamu Igawa; Hajime Imai; Yasuhiro Ogawa; Naoaki Kano; Keita Mamiya; Tomoyo Ikeda; Kiichi Miyamae; Shinji Yamazoe; Jun Torii; Kazuyuki Yamanaka; Toshimasa Kato; Kenta Kawaguchi; Katsuhiro Kawaguchi

doi:10.1111/jce.16037

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

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

Affiliations

¹ Department of Cardiology, Komaki City Hospital, Komaki, Aichi, Japan.
² Department of Cardiovascular Medicine, Nippon Medical School, Tokyo, Japan.

PMID: 37554112
DOI: 10.1111/jce.16037

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.

Keywords: Brugada syndrome; arrhythmogenic right ventricular cardiomyopathy; autopsy; fibrofatty degeneration; ventricular tachycardia.

Publication types

Case Reports

MeSH terms

Aged
Arrhythmias, Cardiac
Arrhythmogenic Right Ventricular Dysplasia* / diagnosis
Brugada Syndrome* / diagnosis
Cardiomegaly
Electrocardiography
Heart Ventricles
Humans
Male
Tachycardia, Ventricular*