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Biventricular Myocardial Fibrosis and Sudden Death in Patients With Brugada Syndrome
BACKGROUND: Electrophysiological, imaging, and pathological studies have reported the presence of subtle structural abnormalities in hearts from patients with Brugada syndrome (BrS). However, data concerning disease involvement outside of the right ventricular outflow tract are limited. OBJECTIVES:...
Autores principales: | , , , , , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Elsevier Biomedical
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8504200/ https://www.ncbi.nlm.nih.gov/pubmed/34620408 http://dx.doi.org/10.1016/j.jacc.2021.08.010 |
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author | Miles, Chris Asimaki, Angeliki Ster, Irina Chis Papadakis, Michael Gray, Belinda Westaby, Joseph Finocchiaro, Gherardo Bueno-Beti, Carlos Ensam, Bode Basu, Joyee Parry-Williams, Gemma MacLachlan, Hamish Edwards, Khari A. Johnson, David Tome, Maite Sharma, Sanjay Sheppard, Mary N. Behr, Elijah R. |
author_facet | Miles, Chris Asimaki, Angeliki Ster, Irina Chis Papadakis, Michael Gray, Belinda Westaby, Joseph Finocchiaro, Gherardo Bueno-Beti, Carlos Ensam, Bode Basu, Joyee Parry-Williams, Gemma MacLachlan, Hamish Edwards, Khari A. Johnson, David Tome, Maite Sharma, Sanjay Sheppard, Mary N. Behr, Elijah R. |
author_sort | Miles, Chris |
collection | PubMed |
description | BACKGROUND: Electrophysiological, imaging, and pathological studies have reported the presence of subtle structural abnormalities in hearts from patients with Brugada syndrome (BrS). However, data concerning disease involvement outside of the right ventricular outflow tract are limited. OBJECTIVES: This study sought to characterize the presence and distribution of ventricular myocardial fibrosis in a cohort of decedents experiencing sudden cardiac death caused by BrS. METHODS: The authors evaluated 28 whole hearts from consecutive sudden cardiac death cases attributed to BrS and 29 hearts from a comparator group comprised of noncardiac deaths (control subjects). Cardiac tissue from 6 regions across the right and left ventricle were stained with Picrosirius red for collagen and tissue composition was determined using image analysis software. Postmortem genetic testing was performed in cases with DNA retained for analysis. RESULTS: Of 28 BrS decedents (75% men; median age of death 25 years), death occurred in sleep or at rest in 24 of 28 (86%). The highest proportion of collagen was observed in the epicardial right ventricular outflow tract of the BrS group (23.7%; 95% CI: 20.8%-26.9%). Ventricular myocardium from BrS decedents demonstrated a higher proportion of collagen compared with control subjects (ratio 1.45; 95% CI: 1.22-1.71; P < 0.001), with no significant interactions with respect to sampling location or tissue layer. There was insufficient evidence to support differences in collagen proportion in SCN5A-positive cases (n = 5) when compared with control subjects (ratio 1.23; 95% CI: 0.75-1.43; P = 0.27). CONCLUSIONS: Brugada syndrome is associated with increased collagen content throughout right and left ventricular myocardium, irrespective of sampling location or myocardial layer. |
format | Online Article Text |
id | pubmed-8504200 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | Elsevier Biomedical |
record_format | MEDLINE/PubMed |
spelling | pubmed-85042002021-10-13 Biventricular Myocardial Fibrosis and Sudden Death in Patients With Brugada Syndrome Miles, Chris Asimaki, Angeliki Ster, Irina Chis Papadakis, Michael Gray, Belinda Westaby, Joseph Finocchiaro, Gherardo Bueno-Beti, Carlos Ensam, Bode Basu, Joyee Parry-Williams, Gemma MacLachlan, Hamish Edwards, Khari A. Johnson, David Tome, Maite Sharma, Sanjay Sheppard, Mary N. Behr, Elijah R. J Am Coll Cardiol Original Investigation BACKGROUND: Electrophysiological, imaging, and pathological studies have reported the presence of subtle structural abnormalities in hearts from patients with Brugada syndrome (BrS). However, data concerning disease involvement outside of the right ventricular outflow tract are limited. OBJECTIVES: This study sought to characterize the presence and distribution of ventricular myocardial fibrosis in a cohort of decedents experiencing sudden cardiac death caused by BrS. METHODS: The authors evaluated 28 whole hearts from consecutive sudden cardiac death cases attributed to BrS and 29 hearts from a comparator group comprised of noncardiac deaths (control subjects). Cardiac tissue from 6 regions across the right and left ventricle were stained with Picrosirius red for collagen and tissue composition was determined using image analysis software. Postmortem genetic testing was performed in cases with DNA retained for analysis. RESULTS: Of 28 BrS decedents (75% men; median age of death 25 years), death occurred in sleep or at rest in 24 of 28 (86%). The highest proportion of collagen was observed in the epicardial right ventricular outflow tract of the BrS group (23.7%; 95% CI: 20.8%-26.9%). Ventricular myocardium from BrS decedents demonstrated a higher proportion of collagen compared with control subjects (ratio 1.45; 95% CI: 1.22-1.71; P < 0.001), with no significant interactions with respect to sampling location or tissue layer. There was insufficient evidence to support differences in collagen proportion in SCN5A-positive cases (n = 5) when compared with control subjects (ratio 1.23; 95% CI: 0.75-1.43; P = 0.27). CONCLUSIONS: Brugada syndrome is associated with increased collagen content throughout right and left ventricular myocardium, irrespective of sampling location or myocardial layer. Elsevier Biomedical 2021-10-12 /pmc/articles/PMC8504200/ /pubmed/34620408 http://dx.doi.org/10.1016/j.jacc.2021.08.010 Text en © 2021 The Authors https://creativecommons.org/licenses/by/4.0/This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Original Investigation Miles, Chris Asimaki, Angeliki Ster, Irina Chis Papadakis, Michael Gray, Belinda Westaby, Joseph Finocchiaro, Gherardo Bueno-Beti, Carlos Ensam, Bode Basu, Joyee Parry-Williams, Gemma MacLachlan, Hamish Edwards, Khari A. Johnson, David Tome, Maite Sharma, Sanjay Sheppard, Mary N. Behr, Elijah R. Biventricular Myocardial Fibrosis and Sudden Death in Patients With Brugada Syndrome |
title | Biventricular Myocardial Fibrosis and Sudden Death in Patients With Brugada Syndrome |
title_full | Biventricular Myocardial Fibrosis and Sudden Death in Patients With Brugada Syndrome |
title_fullStr | Biventricular Myocardial Fibrosis and Sudden Death in Patients With Brugada Syndrome |
title_full_unstemmed | Biventricular Myocardial Fibrosis and Sudden Death in Patients With Brugada Syndrome |
title_short | Biventricular Myocardial Fibrosis and Sudden Death in Patients With Brugada Syndrome |
title_sort | biventricular myocardial fibrosis and sudden death in patients with brugada syndrome |
topic | Original Investigation |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8504200/ https://www.ncbi.nlm.nih.gov/pubmed/34620408 http://dx.doi.org/10.1016/j.jacc.2021.08.010 |
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