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Diagnostic Challenges in Rare Causes of Arrhythmogenic Cardiomyopathy—The Role of Cardiac MRI
Arrhythmogenic right ventricular dysplasia (ARVD) is a rare genetic condition of the myocardium, with a significantly high risk of sudden death. Recent genetic research and improved understanding of the pathophysiology tend to change the ARVD definition towards a larger spectrum of myocardial involv...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8878419/ https://www.ncbi.nlm.nih.gov/pubmed/35207675 http://dx.doi.org/10.3390/jpm12020187 |
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author | Manole, Simona Pintican, Roxana Popa, George Rancea, Raluca Dadarlat-Pop, Alexandra Vulturar, Romana Palade, Emanuel |
author_facet | Manole, Simona Pintican, Roxana Popa, George Rancea, Raluca Dadarlat-Pop, Alexandra Vulturar, Romana Palade, Emanuel |
author_sort | Manole, Simona |
collection | PubMed |
description | Arrhythmogenic right ventricular dysplasia (ARVD) is a rare genetic condition of the myocardium, with a significantly high risk of sudden death. Recent genetic research and improved understanding of the pathophysiology tend to change the ARVD definition towards a larger spectrum of myocardial involvement, which includes, in various proportions, both the right (RV) and left ventricle (LV), currently referred to as ACM (arrhythmogenic cardiomyopathy). Its pathological substrate is defined by the replacement of the ventricular myocardium with fibrous adipose tissue that further leads to inadequate electrical impulses and translates into varies degrees of malignant ventricular arrythmias and dyskinetic myocardium movements. Particularly, the cardio-cutaneous syndromes of Carvajal/Naxos represent rare causes of ACM that might be suspected from early childhood. The diagnostic is sometimes challenging, even with well-established rTFC or Padua criteria, especially for pediatric patients or ACM with LV involvement. Cardiac MRI gain more and more importance in ACM diagnostic especially in non-classical forms. Furthermore, MRI is useful in highlighting myocardial fibrosis, fatty replacement or wall movement with high accuracy, thus guiding not only the depiction, but also the patient’s stratification and management. |
format | Online Article Text |
id | pubmed-8878419 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-88784192022-02-26 Diagnostic Challenges in Rare Causes of Arrhythmogenic Cardiomyopathy—The Role of Cardiac MRI Manole, Simona Pintican, Roxana Popa, George Rancea, Raluca Dadarlat-Pop, Alexandra Vulturar, Romana Palade, Emanuel J Pers Med Review Arrhythmogenic right ventricular dysplasia (ARVD) is a rare genetic condition of the myocardium, with a significantly high risk of sudden death. Recent genetic research and improved understanding of the pathophysiology tend to change the ARVD definition towards a larger spectrum of myocardial involvement, which includes, in various proportions, both the right (RV) and left ventricle (LV), currently referred to as ACM (arrhythmogenic cardiomyopathy). Its pathological substrate is defined by the replacement of the ventricular myocardium with fibrous adipose tissue that further leads to inadequate electrical impulses and translates into varies degrees of malignant ventricular arrythmias and dyskinetic myocardium movements. Particularly, the cardio-cutaneous syndromes of Carvajal/Naxos represent rare causes of ACM that might be suspected from early childhood. The diagnostic is sometimes challenging, even with well-established rTFC or Padua criteria, especially for pediatric patients or ACM with LV involvement. Cardiac MRI gain more and more importance in ACM diagnostic especially in non-classical forms. Furthermore, MRI is useful in highlighting myocardial fibrosis, fatty replacement or wall movement with high accuracy, thus guiding not only the depiction, but also the patient’s stratification and management. MDPI 2022-01-31 /pmc/articles/PMC8878419/ /pubmed/35207675 http://dx.doi.org/10.3390/jpm12020187 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 Manole, Simona Pintican, Roxana Popa, George Rancea, Raluca Dadarlat-Pop, Alexandra Vulturar, Romana Palade, Emanuel Diagnostic Challenges in Rare Causes of Arrhythmogenic Cardiomyopathy—The Role of Cardiac MRI |
title | Diagnostic Challenges in Rare Causes of Arrhythmogenic Cardiomyopathy—The Role of Cardiac MRI |
title_full | Diagnostic Challenges in Rare Causes of Arrhythmogenic Cardiomyopathy—The Role of Cardiac MRI |
title_fullStr | Diagnostic Challenges in Rare Causes of Arrhythmogenic Cardiomyopathy—The Role of Cardiac MRI |
title_full_unstemmed | Diagnostic Challenges in Rare Causes of Arrhythmogenic Cardiomyopathy—The Role of Cardiac MRI |
title_short | Diagnostic Challenges in Rare Causes of Arrhythmogenic Cardiomyopathy—The Role of Cardiac MRI |
title_sort | diagnostic challenges in rare causes of arrhythmogenic cardiomyopathy—the role of cardiac mri |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8878419/ https://www.ncbi.nlm.nih.gov/pubmed/35207675 http://dx.doi.org/10.3390/jpm12020187 |
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