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Sudden cardiac death owing to arrhythmogenic right ventricular cardiomyopathy: Two case reports and systematic literature review

BACKGROUND AND OBJECTIVE: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is increasingly recognized in forensic practice with controversial diagnosis. Here we described the epidemiological characteristics and reported the pathogenetic mechanism, diagnostic challenges, and forensic implicatio...

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Autores principales: Mu, Jiao, Zhang, Guohui, Xue, Dazhong, Xi, Mengrou, Qi, Jiarui, Dong, Hongmei
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer Health 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5708984/
https://www.ncbi.nlm.nih.gov/pubmed/29381985
http://dx.doi.org/10.1097/MD.0000000000008808
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author Mu, Jiao
Zhang, Guohui
Xue, Dazhong
Xi, Mengrou
Qi, Jiarui
Dong, Hongmei
author_facet Mu, Jiao
Zhang, Guohui
Xue, Dazhong
Xi, Mengrou
Qi, Jiarui
Dong, Hongmei
author_sort Mu, Jiao
collection PubMed
description BACKGROUND AND OBJECTIVE: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is increasingly recognized in forensic practice with controversial diagnosis. Here we described the epidemiological characteristics and reported the pathogenetic mechanism, diagnostic challenges, and forensic implications of Chinese ARVC autopsy cases. METHODS: Two cases of sudden cardiac death owing to ARVC were reported. Retrospective analysis were performed on such 2 cases and 45 cases of separate ARVC complete autopsy case reports through Chinese literature databases in the last 30 years. RESULTS: There were 27 males and 20 females, and the mean age at death was 35 years. Sudden cardiac death was the first manifestation observed in most patients, with no previous family and medical history. Exercise, acute stress, increased cardiac workload, and ethanol are frequently involved. The mean heart weight was 393 g (range, 240–590 g), and 10 cases had relative heart hypertrophy. Microscopic abnormalities included replacement of myocardium by adipose infiltration in 68.09% cases and fibroadipose in 31.91% cases; 80.85% cases were restricted to the right ventricle (RV), whereas biventricular subtype was seen in the remaining 19.15% cases. The preliminary quantitative histology showed 60.7% of fat tissues, 12.1% of fibrosis, and 27.2% residual myocytes in RV. Inflammatory cell infiltration was found in 25.53% cases, but myocyte necrosis was found in only 1 case. In 10.64% of cases, cardiac conduction was infiltrated by fibrosis, adipose, or both. CONCLUSION: In this review, the most characteristic and distinct histopathologic features that are diagnostic or highly suggestive of ARVC for forensic pathologists were identified. Combining gross and histological examinations with postmortem genetic analysis is recommended for identifying ARVC.
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spelling pubmed-57089842017-12-07 Sudden cardiac death owing to arrhythmogenic right ventricular cardiomyopathy: Two case reports and systematic literature review Mu, Jiao Zhang, Guohui Xue, Dazhong Xi, Mengrou Qi, Jiarui Dong, Hongmei Medicine (Baltimore) 3400 BACKGROUND AND OBJECTIVE: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is increasingly recognized in forensic practice with controversial diagnosis. Here we described the epidemiological characteristics and reported the pathogenetic mechanism, diagnostic challenges, and forensic implications of Chinese ARVC autopsy cases. METHODS: Two cases of sudden cardiac death owing to ARVC were reported. Retrospective analysis were performed on such 2 cases and 45 cases of separate ARVC complete autopsy case reports through Chinese literature databases in the last 30 years. RESULTS: There were 27 males and 20 females, and the mean age at death was 35 years. Sudden cardiac death was the first manifestation observed in most patients, with no previous family and medical history. Exercise, acute stress, increased cardiac workload, and ethanol are frequently involved. The mean heart weight was 393 g (range, 240–590 g), and 10 cases had relative heart hypertrophy. Microscopic abnormalities included replacement of myocardium by adipose infiltration in 68.09% cases and fibroadipose in 31.91% cases; 80.85% cases were restricted to the right ventricle (RV), whereas biventricular subtype was seen in the remaining 19.15% cases. The preliminary quantitative histology showed 60.7% of fat tissues, 12.1% of fibrosis, and 27.2% residual myocytes in RV. Inflammatory cell infiltration was found in 25.53% cases, but myocyte necrosis was found in only 1 case. In 10.64% of cases, cardiac conduction was infiltrated by fibrosis, adipose, or both. CONCLUSION: In this review, the most characteristic and distinct histopathologic features that are diagnostic or highly suggestive of ARVC for forensic pathologists were identified. Combining gross and histological examinations with postmortem genetic analysis is recommended for identifying ARVC. Wolters Kluwer Health 2017-11-27 /pmc/articles/PMC5708984/ /pubmed/29381985 http://dx.doi.org/10.1097/MD.0000000000008808 Text en Copyright © 2017 the Author(s). Published by Wolters Kluwer Health, Inc. http://creativecommons.org/licenses/by/4.0 This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. http://creativecommons.org/licenses/by/4.0
spellingShingle 3400
Mu, Jiao
Zhang, Guohui
Xue, Dazhong
Xi, Mengrou
Qi, Jiarui
Dong, Hongmei
Sudden cardiac death owing to arrhythmogenic right ventricular cardiomyopathy: Two case reports and systematic literature review
title Sudden cardiac death owing to arrhythmogenic right ventricular cardiomyopathy: Two case reports and systematic literature review
title_full Sudden cardiac death owing to arrhythmogenic right ventricular cardiomyopathy: Two case reports and systematic literature review
title_fullStr Sudden cardiac death owing to arrhythmogenic right ventricular cardiomyopathy: Two case reports and systematic literature review
title_full_unstemmed Sudden cardiac death owing to arrhythmogenic right ventricular cardiomyopathy: Two case reports and systematic literature review
title_short Sudden cardiac death owing to arrhythmogenic right ventricular cardiomyopathy: Two case reports and systematic literature review
title_sort sudden cardiac death owing to arrhythmogenic right ventricular cardiomyopathy: two case reports and systematic literature review
topic 3400
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5708984/
https://www.ncbi.nlm.nih.gov/pubmed/29381985
http://dx.doi.org/10.1097/MD.0000000000008808
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