Autoptic findings of sudden cardiac death (SCD) in patients with arrhythmogenic ventricular cardiomiopathy (AVC) from left ventricle and biventricular involvement.
ARVC
AVC
Arrhythmogenic right ventricular cardiomyopathy
Arrhythmogenic ventricular cardiomyopathy
Autopsy study
Fibrofatty replacement
Histology criteria
Sudden cardiac death
Journal
Pathology, research and practice
ISSN: 1618-0631
Titre abrégé: Pathol Res Pract
Pays: Germany
ID NLM: 7806109
Informations de publication
Date de publication:
Dec 2020
Dec 2020
Historique:
received:
23
09
2020
revised:
25
10
2020
accepted:
26
10
2020
pubmed:
12
11
2020
medline:
29
9
2021
entrez:
11
11
2020
Statut:
ppublish
Résumé
To evaluate autoptic histopathological findings of arrhythmogenic ventricular cardiomyopathy (AVC) as major cause of sudden cardiac death (SCD) in young adults. According to Heart Rhythm Society (HRS)'s international consensus, histological criteria for AVC diagnosis include a progressive myocardial atrophy of the right ventricle characterized by a transmural fatty or fibrofatty replacement in a segmental or diffuse pattern (residual myocytes <60 % vs 60-75 % by morphometric analysis) explaining the electrical instability with increased risk of SCD. However, there is increasing evidence for atypical patterns of localizations and percentage of fibrofatty replacement suggesting the need to update histopathological features of AVC. Histology examination of ventricles, atria, and septum was performed on 10 autopsy of SCD due to AVC. Staining with hematoxylin-eosin and PicroSirius Red/Fast Green were performed on the heart samples to identify specific fibrofatty patterns. Our analysis showed that: 1) myocardial replacement by a diffuse segmental fatty or fibro-fatty tissue characterized right and left ventricles as well as atrial walls; 2) the degree of fibrofatty tissue replacement was less than 40 % both in left ventricle (n = 4, 40 %) and biventricular (n = 6, 60 %) localization; 3) perivascular fibrosis, inflammatory infiltrate, areas of hypertrophy and/or areas of coagulative necrosis as signs of hypoxic damage in the first stage. We confirmed prior evidence for fibrofatty replacement both in biventricular and septal localizations. Importantly, we observed a less degree (<40 %) of fibrofatty replacement as compared to current guidelines. This supports the need to further explore the histological patterns of fibrofatty infiltration in a larger study population to improve the histological diagnostic criteria of AVC.
Sections du résumé
OBJECTIVES
OBJECTIVE
To evaluate autoptic histopathological findings of arrhythmogenic ventricular cardiomyopathy (AVC) as major cause of sudden cardiac death (SCD) in young adults.
BACKGROUND
BACKGROUND
According to Heart Rhythm Society (HRS)'s international consensus, histological criteria for AVC diagnosis include a progressive myocardial atrophy of the right ventricle characterized by a transmural fatty or fibrofatty replacement in a segmental or diffuse pattern (residual myocytes <60 % vs 60-75 % by morphometric analysis) explaining the electrical instability with increased risk of SCD. However, there is increasing evidence for atypical patterns of localizations and percentage of fibrofatty replacement suggesting the need to update histopathological features of AVC.
METHODS
METHODS
Histology examination of ventricles, atria, and septum was performed on 10 autopsy of SCD due to AVC. Staining with hematoxylin-eosin and PicroSirius Red/Fast Green were performed on the heart samples to identify specific fibrofatty patterns.
RESULTS
RESULTS
Our analysis showed that: 1) myocardial replacement by a diffuse segmental fatty or fibro-fatty tissue characterized right and left ventricles as well as atrial walls; 2) the degree of fibrofatty tissue replacement was less than 40 % both in left ventricle (n = 4, 40 %) and biventricular (n = 6, 60 %) localization; 3) perivascular fibrosis, inflammatory infiltrate, areas of hypertrophy and/or areas of coagulative necrosis as signs of hypoxic damage in the first stage.
CONCLUSIONS
CONCLUSIONS
We confirmed prior evidence for fibrofatty replacement both in biventricular and septal localizations. Importantly, we observed a less degree (<40 %) of fibrofatty replacement as compared to current guidelines. This supports the need to further explore the histological patterns of fibrofatty infiltration in a larger study population to improve the histological diagnostic criteria of AVC.
Identifiants
pubmed: 33176260
pii: S0344-0338(20)32124-5
doi: 10.1016/j.prp.2020.153269
pii:
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
153269Informations de copyright
Copyright © 2020 The Authors. Published by Elsevier GmbH.. All rights reserved.