Cargando…

Myocarditis-like Episodes in Patients with Arrhythmogenic Cardiomyopathy: A Systematic Review on the So-Called Hot-Phase of the Disease

Arrhythmogenic cardiomyopathy (ACM) is a genetically determined myocardial disease, characterized by myocytes necrosis with fibrofatty substitution and ventricular arrhythmias that can even lead to sudden cardiac death. The presence of inflammatory cell infiltrates in endomyocardial biopsies or in a...

Descripción completa

Detalles Bibliográficos
Autores principales: Bariani, Riccardo, Rigato, Ilaria, Cipriani, Alberto, Bueno Marinas, Maria, Celeghin, Rudy, Basso, Cristina, Corrado, Domenico, Pilichou, Kalliopi, Bauce, Barbara
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9496041/
https://www.ncbi.nlm.nih.gov/pubmed/36139162
http://dx.doi.org/10.3390/biom12091324
_version_ 1784794171048984576
author Bariani, Riccardo
Rigato, Ilaria
Cipriani, Alberto
Bueno Marinas, Maria
Celeghin, Rudy
Basso, Cristina
Corrado, Domenico
Pilichou, Kalliopi
Bauce, Barbara
author_facet Bariani, Riccardo
Rigato, Ilaria
Cipriani, Alberto
Bueno Marinas, Maria
Celeghin, Rudy
Basso, Cristina
Corrado, Domenico
Pilichou, Kalliopi
Bauce, Barbara
author_sort Bariani, Riccardo
collection PubMed
description Arrhythmogenic cardiomyopathy (ACM) is a genetically determined myocardial disease, characterized by myocytes necrosis with fibrofatty substitution and ventricular arrhythmias that can even lead to sudden cardiac death. The presence of inflammatory cell infiltrates in endomyocardial biopsies or in autoptic specimens of ACM patients has been reported, suggesting a possible role of inflammation in the pathophysiology of the disease. Furthermore, chest pain episodes accompanied by electrocardiographic changes and troponin release have been observed and defined as the “hot-phase” phenomenon. The aim of this critical systematic review was to assess the clinical features of ACM patients presenting with “hot-phase” episodes. According to PRISMA guidelines, a search was run in the PubMed, Scopus and Web of Science electronic databases using the following keywords: “arrhythmogenic cardiomyopathy”; “myocarditis” or “arrhythmogenic cardiomyopathy”; “troponin” or “arrhythmogenic cardiomyopathy”; and “hot-phase”. A total of 1433 titles were retrieved, of which 65 studies were potentially relevant to the topic. Through the application of inclusion and exclusion criteria, 9 papers reporting 103 ACM patients who had experienced hot-phase episodes were selected for this review. Age at time of episodes was available in 76% of cases, with the mean age reported being 26 years ± 14 years (min 2–max 71 years). Overall, 86% of patients showed left ventricular epicardial LGE. At the time of hot-phase episodes, 49% received a diagnosis of ACM (Arrhythmogenic left ventricular cardiomyopathy in the majority of cases), 19% of dilated cardiomyopathy and 26% of acute myocarditis. At the genetic study, Desmoplakin (DSP) was the more represented disease-gene (69%), followed by Plakophillin-2 (9%) and Desmoglein-2 (6%). In conclusion, ACM patients showing hot-phase episodes are usually young, and DSP is the most common disease gene, accounting for 69% of cases. Currently, the role of “hot-phase” episodes in disease progression and arrhythmic risk stratification remains to be clarified.
format Online
Article
Text
id pubmed-9496041
institution National Center for Biotechnology Information
language English
publishDate 2022
publisher MDPI
record_format MEDLINE/PubMed
spelling pubmed-94960412022-09-23 Myocarditis-like Episodes in Patients with Arrhythmogenic Cardiomyopathy: A Systematic Review on the So-Called Hot-Phase of the Disease Bariani, Riccardo Rigato, Ilaria Cipriani, Alberto Bueno Marinas, Maria Celeghin, Rudy Basso, Cristina Corrado, Domenico Pilichou, Kalliopi Bauce, Barbara Biomolecules Review Arrhythmogenic cardiomyopathy (ACM) is a genetically determined myocardial disease, characterized by myocytes necrosis with fibrofatty substitution and ventricular arrhythmias that can even lead to sudden cardiac death. The presence of inflammatory cell infiltrates in endomyocardial biopsies or in autoptic specimens of ACM patients has been reported, suggesting a possible role of inflammation in the pathophysiology of the disease. Furthermore, chest pain episodes accompanied by electrocardiographic changes and troponin release have been observed and defined as the “hot-phase” phenomenon. The aim of this critical systematic review was to assess the clinical features of ACM patients presenting with “hot-phase” episodes. According to PRISMA guidelines, a search was run in the PubMed, Scopus and Web of Science electronic databases using the following keywords: “arrhythmogenic cardiomyopathy”; “myocarditis” or “arrhythmogenic cardiomyopathy”; “troponin” or “arrhythmogenic cardiomyopathy”; and “hot-phase”. A total of 1433 titles were retrieved, of which 65 studies were potentially relevant to the topic. Through the application of inclusion and exclusion criteria, 9 papers reporting 103 ACM patients who had experienced hot-phase episodes were selected for this review. Age at time of episodes was available in 76% of cases, with the mean age reported being 26 years ± 14 years (min 2–max 71 years). Overall, 86% of patients showed left ventricular epicardial LGE. At the time of hot-phase episodes, 49% received a diagnosis of ACM (Arrhythmogenic left ventricular cardiomyopathy in the majority of cases), 19% of dilated cardiomyopathy and 26% of acute myocarditis. At the genetic study, Desmoplakin (DSP) was the more represented disease-gene (69%), followed by Plakophillin-2 (9%) and Desmoglein-2 (6%). In conclusion, ACM patients showing hot-phase episodes are usually young, and DSP is the most common disease gene, accounting for 69% of cases. Currently, the role of “hot-phase” episodes in disease progression and arrhythmic risk stratification remains to be clarified. MDPI 2022-09-19 /pmc/articles/PMC9496041/ /pubmed/36139162 http://dx.doi.org/10.3390/biom12091324 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Bariani, Riccardo
Rigato, Ilaria
Cipriani, Alberto
Bueno Marinas, Maria
Celeghin, Rudy
Basso, Cristina
Corrado, Domenico
Pilichou, Kalliopi
Bauce, Barbara
Myocarditis-like Episodes in Patients with Arrhythmogenic Cardiomyopathy: A Systematic Review on the So-Called Hot-Phase of the Disease
title Myocarditis-like Episodes in Patients with Arrhythmogenic Cardiomyopathy: A Systematic Review on the So-Called Hot-Phase of the Disease
title_full Myocarditis-like Episodes in Patients with Arrhythmogenic Cardiomyopathy: A Systematic Review on the So-Called Hot-Phase of the Disease
title_fullStr Myocarditis-like Episodes in Patients with Arrhythmogenic Cardiomyopathy: A Systematic Review on the So-Called Hot-Phase of the Disease
title_full_unstemmed Myocarditis-like Episodes in Patients with Arrhythmogenic Cardiomyopathy: A Systematic Review on the So-Called Hot-Phase of the Disease
title_short Myocarditis-like Episodes in Patients with Arrhythmogenic Cardiomyopathy: A Systematic Review on the So-Called Hot-Phase of the Disease
title_sort myocarditis-like episodes in patients with arrhythmogenic cardiomyopathy: a systematic review on the so-called hot-phase of the disease
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9496041/
https://www.ncbi.nlm.nih.gov/pubmed/36139162
http://dx.doi.org/10.3390/biom12091324
work_keys_str_mv AT barianiriccardo myocarditislikeepisodesinpatientswitharrhythmogeniccardiomyopathyasystematicreviewonthesocalledhotphaseofthedisease
AT rigatoilaria myocarditislikeepisodesinpatientswitharrhythmogeniccardiomyopathyasystematicreviewonthesocalledhotphaseofthedisease
AT ciprianialberto myocarditislikeepisodesinpatientswitharrhythmogeniccardiomyopathyasystematicreviewonthesocalledhotphaseofthedisease
AT buenomarinasmaria myocarditislikeepisodesinpatientswitharrhythmogeniccardiomyopathyasystematicreviewonthesocalledhotphaseofthedisease
AT celeghinrudy myocarditislikeepisodesinpatientswitharrhythmogeniccardiomyopathyasystematicreviewonthesocalledhotphaseofthedisease
AT bassocristina myocarditislikeepisodesinpatientswitharrhythmogeniccardiomyopathyasystematicreviewonthesocalledhotphaseofthedisease
AT corradodomenico myocarditislikeepisodesinpatientswitharrhythmogeniccardiomyopathyasystematicreviewonthesocalledhotphaseofthedisease
AT pilichoukalliopi myocarditislikeepisodesinpatientswitharrhythmogeniccardiomyopathyasystematicreviewonthesocalledhotphaseofthedisease
AT baucebarbara myocarditislikeepisodesinpatientswitharrhythmogeniccardiomyopathyasystematicreviewonthesocalledhotphaseofthedisease