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Myocardial Function, Heart Failure and Arrhythmia in Marfan Syndrome: A Systematic Literature Review
Marfan syndrome (MFS) is a heritable systemic connective tissue disease with important cardiovascular involvement, including aortic root dilatation and mitral valve prolapse. Life expectancy in patients with MFS is mainly determined by cardiovascular complications, among which aortic dissection or r...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7599866/ https://www.ncbi.nlm.nih.gov/pubmed/32992882 http://dx.doi.org/10.3390/diagnostics10100751 |
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author | Demolder, Anthony von Kodolitsch, Yskert Muiño-Mosquera, Laura De Backer, Julie |
author_facet | Demolder, Anthony von Kodolitsch, Yskert Muiño-Mosquera, Laura De Backer, Julie |
author_sort | Demolder, Anthony |
collection | PubMed |
description | Marfan syndrome (MFS) is a heritable systemic connective tissue disease with important cardiovascular involvement, including aortic root dilatation and mitral valve prolapse. Life expectancy in patients with MFS is mainly determined by cardiovascular complications, among which aortic dissection or rupture are most dreaded. In recent years, heart failure and ventricular arrhythmia have drawn attention as extra-aortic cardiovascular manifestations and as additional reported causes of death. Imaging studies have provided data supporting a primary myocardial impairment in the absence of valvular disease or cardiovascular surgery, while studies using ambulatory ECG have demonstrated an increased susceptibility to ventricular arrhythmia. In this paper, current literature was reviewed in order to provide insights in characteristics, pathophysiology and evolution of myocardial function, heart failure and ventricular arrhythmia in MFS. |
format | Online Article Text |
id | pubmed-7599866 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-75998662020-11-01 Myocardial Function, Heart Failure and Arrhythmia in Marfan Syndrome: A Systematic Literature Review Demolder, Anthony von Kodolitsch, Yskert Muiño-Mosquera, Laura De Backer, Julie Diagnostics (Basel) Review Marfan syndrome (MFS) is a heritable systemic connective tissue disease with important cardiovascular involvement, including aortic root dilatation and mitral valve prolapse. Life expectancy in patients with MFS is mainly determined by cardiovascular complications, among which aortic dissection or rupture are most dreaded. In recent years, heart failure and ventricular arrhythmia have drawn attention as extra-aortic cardiovascular manifestations and as additional reported causes of death. Imaging studies have provided data supporting a primary myocardial impairment in the absence of valvular disease or cardiovascular surgery, while studies using ambulatory ECG have demonstrated an increased susceptibility to ventricular arrhythmia. In this paper, current literature was reviewed in order to provide insights in characteristics, pathophysiology and evolution of myocardial function, heart failure and ventricular arrhythmia in MFS. MDPI 2020-09-25 /pmc/articles/PMC7599866/ /pubmed/32992882 http://dx.doi.org/10.3390/diagnostics10100751 Text en © 2020 by the authors. 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 (http://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Review Demolder, Anthony von Kodolitsch, Yskert Muiño-Mosquera, Laura De Backer, Julie Myocardial Function, Heart Failure and Arrhythmia in Marfan Syndrome: A Systematic Literature Review |
title | Myocardial Function, Heart Failure and Arrhythmia in Marfan Syndrome: A Systematic Literature Review |
title_full | Myocardial Function, Heart Failure and Arrhythmia in Marfan Syndrome: A Systematic Literature Review |
title_fullStr | Myocardial Function, Heart Failure and Arrhythmia in Marfan Syndrome: A Systematic Literature Review |
title_full_unstemmed | Myocardial Function, Heart Failure and Arrhythmia in Marfan Syndrome: A Systematic Literature Review |
title_short | Myocardial Function, Heart Failure and Arrhythmia in Marfan Syndrome: A Systematic Literature Review |
title_sort | myocardial function, heart failure and arrhythmia in marfan syndrome: a systematic literature review |
topic | Review |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7599866/ https://www.ncbi.nlm.nih.gov/pubmed/32992882 http://dx.doi.org/10.3390/diagnostics10100751 |
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