Structural Heart Alterations in Brugada Syndrome: Is it Really a Channelopathy? A Systematic Review.

Brugada syndrome endomyocardial biopsy forensic pathology histopathology sudden cardiac death

Journal

Journal of clinical medicine
ISSN: 2077-0383
Titre abrégé: J Clin Med
Pays: Switzerland
ID NLM: 101606588

Informations de publication

Date de publication:
28 Jul 2022
Historique:
received: 12 06 2022
revised: 26 07 2022
accepted: 27 07 2022
entrez: 12 8 2022
pubmed: 13 8 2022
medline: 13 8 2022
Statut: epublish

Résumé

Brugada syndrome (BrS) is classified as an inherited cardiac channelopathy attributed to dysfunctional ion channels and/or associated proteins in cardiomyocytes rather than to structural heart alterations. However, hearts of some BrS patients exhibit slight histologic abnormalities, suggesting that BrS could be a phenotypic variant of arrhythmogenic cardiomyopathy. We performed a systematic review of the literature following Preferred Reporting Items for Systematic Reviews and Meta-Analyses Statement (PRISMA) criteria. Our comprehensive analysis of structural findings did not reveal enough definitive evidence for reclassification of BrS as a cardiomyopathy. The collection and comprehensive analysis of new cases with a definitive BrS diagnosis are needed to clarify whether some of these structural features may have key roles in the pathophysiological pathways associated with malignant arrhythmogenic episodes.

Identifiants

pubmed: 35956023
pii: jcm11154406
doi: 10.3390/jcm11154406
pmc: PMC9368908
pii:
doi:

Types de publication

Journal Article Review

Langues

eng

Subventions

Organisme : La Caixa Foundation
ID : LCF/PR/GN16/50290001
Organisme : La Caixa Foundation
ID : LCF/PR/GN19/50320002
Organisme : Fundacio Marato TV3
ID : 245/U/2020

Déclaration de conflit d'intérêts

The authors declare no conflict of interest.

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Auteurs

Antonio Oliva (A)

Department of Health Surveillance and Bioethics, Section of Legal Medicine, Fondazione Policlinico A. Gemelli IRCCS, Università Cattolica del Sacro Cuore, 00168 Rome, Italy.

Simone Grassi (S)

Department of Health Sciences, Section of Forensic Medical Sciences, University of Florence, Largo Brambilla 3, 50134 Florence, Italy.

Vilma Pinchi (V)

Department of Health Sciences, Section of Forensic Medical Sciences, University of Florence, Largo Brambilla 3, 50134 Florence, Italy.

Francesca Cazzato (F)

Department of Health Surveillance and Bioethics, Section of Legal Medicine, Fondazione Policlinico A. Gemelli IRCCS, Università Cattolica del Sacro Cuore, 00168 Rome, Italy.

Mónica Coll (M)

Cardiovascular Genetics Center, University of Girona-IDIBGI, 17190 Girona, Spain.
Centro de Investigación Biomédica en Red. Enfermedades Cardiovasculares (CIBERCV), 28029 Madrid, Spain.

Mireia Alcalde (M)

Cardiovascular Genetics Center, University of Girona-IDIBGI, 17190 Girona, Spain.

Marta Vallverdú-Prats (M)

Cardiovascular Genetics Center, University of Girona-IDIBGI, 17190 Girona, Spain.

Alexandra Perez-Serra (A)

Cardiovascular Genetics Center, University of Girona-IDIBGI, 17190 Girona, Spain.

Estefanía Martínez-Barrios (E)

Pediatric Arrhythmias, Inherited Cardiac Diseases and Sudden Death Unit, Cardiology Department, Sant Joan de Déu Hospital de Barcelona, 08950 Barcelona, Spain.
European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart (ERN GUARD-Heart), 1105 AZ Amsterdam, The Netherlands.
Arrítmies Pediàtriques, Cardiologia Genètica i Mort Sobtada, Malalties Cardiovasculars en el Desenvolupament, Institut de Recerca Sant Joan de Déu, Esplugues de Llobregat, 08950 Barcelona, Spain.

Sergi Cesar (S)

Pediatric Arrhythmias, Inherited Cardiac Diseases and Sudden Death Unit, Cardiology Department, Sant Joan de Déu Hospital de Barcelona, 08950 Barcelona, Spain.
European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart (ERN GUARD-Heart), 1105 AZ Amsterdam, The Netherlands.
Arrítmies Pediàtriques, Cardiologia Genètica i Mort Sobtada, Malalties Cardiovasculars en el Desenvolupament, Institut de Recerca Sant Joan de Déu, Esplugues de Llobregat, 08950 Barcelona, Spain.

Anna Iglesias (A)

Cardiovascular Genetics Center, University of Girona-IDIBGI, 17190 Girona, Spain.

José Cruzalegui (J)

Pediatric Arrhythmias, Inherited Cardiac Diseases and Sudden Death Unit, Cardiology Department, Sant Joan de Déu Hospital de Barcelona, 08950 Barcelona, Spain.
European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart (ERN GUARD-Heart), 1105 AZ Amsterdam, The Netherlands.
Arrítmies Pediàtriques, Cardiologia Genètica i Mort Sobtada, Malalties Cardiovasculars en el Desenvolupament, Institut de Recerca Sant Joan de Déu, Esplugues de Llobregat, 08950 Barcelona, Spain.

Clara Hernández (C)

Pediatric Arrhythmias, Inherited Cardiac Diseases and Sudden Death Unit, Cardiology Department, Sant Joan de Déu Hospital de Barcelona, 08950 Barcelona, Spain.

Victoria Fiol (V)

Pediatric Arrhythmias, Inherited Cardiac Diseases and Sudden Death Unit, Cardiology Department, Sant Joan de Déu Hospital de Barcelona, 08950 Barcelona, Spain.
European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart (ERN GUARD-Heart), 1105 AZ Amsterdam, The Netherlands.
Arrítmies Pediàtriques, Cardiologia Genètica i Mort Sobtada, Malalties Cardiovasculars en el Desenvolupament, Institut de Recerca Sant Joan de Déu, Esplugues de Llobregat, 08950 Barcelona, Spain.

Elena Arbelo (E)

Centro de Investigación Biomédica en Red. Enfermedades Cardiovasculares (CIBERCV), 28029 Madrid, Spain.
European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart (ERN GUARD-Heart), 1105 AZ Amsterdam, The Netherlands.
Arrítmies Pediàtriques, Cardiologia Genètica i Mort Sobtada, Malalties Cardiovasculars en el Desenvolupament, Institut de Recerca Sant Joan de Déu, Esplugues de Llobregat, 08950 Barcelona, Spain.

Nuria Díez-Escuté (N)

Arrhythmias Unit, Hospital Clinic, University of Barcelona-IDIBAPS, 08036 Barcelona, Spain.

Vincenzo Arena (V)

Institute of Pathological Anatomy, School of Medicine, Catholic University, 00168 Rome, Italy.

Josep Brugada (J)

Centro de Investigación Biomédica en Red. Enfermedades Cardiovasculares (CIBERCV), 28029 Madrid, Spain.
Pediatric Arrhythmias, Inherited Cardiac Diseases and Sudden Death Unit, Cardiology Department, Sant Joan de Déu Hospital de Barcelona, 08950 Barcelona, Spain.
European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart (ERN GUARD-Heart), 1105 AZ Amsterdam, The Netherlands.
Arrítmies Pediàtriques, Cardiologia Genètica i Mort Sobtada, Malalties Cardiovasculars en el Desenvolupament, Institut de Recerca Sant Joan de Déu, Esplugues de Llobregat, 08950 Barcelona, Spain.
Arrhythmias Unit, Hospital Clinic, University of Barcelona-IDIBAPS, 08036 Barcelona, Spain.

Georgia Sarquella-Brugada (G)

Pediatric Arrhythmias, Inherited Cardiac Diseases and Sudden Death Unit, Cardiology Department, Sant Joan de Déu Hospital de Barcelona, 08950 Barcelona, Spain.
European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart (ERN GUARD-Heart), 1105 AZ Amsterdam, The Netherlands.
Arrítmies Pediàtriques, Cardiologia Genètica i Mort Sobtada, Malalties Cardiovasculars en el Desenvolupament, Institut de Recerca Sant Joan de Déu, Esplugues de Llobregat, 08950 Barcelona, Spain.
Medical Science Department, School of Medicine, University of Girona, 17003 Girona, Spain.

Ramon Brugada (R)

Cardiovascular Genetics Center, University of Girona-IDIBGI, 17190 Girona, Spain.
Centro de Investigación Biomédica en Red. Enfermedades Cardiovasculares (CIBERCV), 28029 Madrid, Spain.
Institute of Pathological Anatomy, School of Medicine, Catholic University, 00168 Rome, Italy.
Cardiology Service, Hospital Josep Trueta, University of Girona, 17007 Girona, Spain.

Oscar Campuzano (O)

Cardiovascular Genetics Center, University of Girona-IDIBGI, 17190 Girona, Spain.
Centro de Investigación Biomédica en Red. Enfermedades Cardiovasculares (CIBERCV), 28029 Madrid, Spain.
Medical Science Department, School of Medicine, University of Girona, 17003 Girona, Spain.

Classifications MeSH