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Unravelling the Pathogenetic Mechanisms in Congenital Aortopathies: Need for an Integrative Translational Approach

Congenital heart disease (CHD)-associated aortopathy is a very heterogeneous entity with a wide spectrum of clinical presentations. The pathogenesis of aortopathy is still incompletely understood, and, therefore, the best prevention and management strategy is currently unknown. The most common entit...

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Autores principales: Girdauskas, Evaldas, Kaemmerer, Harald, von Kodolitsch, Yskert
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7019613/
https://www.ncbi.nlm.nih.gov/pubmed/31940858
http://dx.doi.org/10.3390/jcm9010204
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author Girdauskas, Evaldas
Kaemmerer, Harald
von Kodolitsch, Yskert
author_facet Girdauskas, Evaldas
Kaemmerer, Harald
von Kodolitsch, Yskert
author_sort Girdauskas, Evaldas
collection PubMed
description Congenital heart disease (CHD)-associated aortopathy is a very heterogeneous entity with a wide spectrum of clinical presentations. The pathogenesis of aortopathy is still incompletely understood, and, therefore, the best prevention and management strategy is currently unknown. The most common entity of CHD-associated aortopathies is bicuspid aortic valve (BAV)-associated aortic disease (so called bicuspid aortopathy) that is found in 50%–60% of BAV individuals. BAV aortopathy has been reported in association with an increased risk of aortic events, especially aortic dissection and sudden cardiac death. Risk stratification of adverse aortic events is still very rudimentary and considers only the maximal aortic diameter, which makes it unsuitable for an individual risk prediction. This introductory Editorial highlights the unmet clinical need for more integrative and translational research to unravel pathogenetic pathways in the development of CHD-associated aortopathies, integrating recently identified genetic lesions and knowledge on circulating biomarkers and microstructural changes in the diseased aorta.
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spelling pubmed-70196132020-03-09 Unravelling the Pathogenetic Mechanisms in Congenital Aortopathies: Need for an Integrative Translational Approach Girdauskas, Evaldas Kaemmerer, Harald von Kodolitsch, Yskert J Clin Med Editorial Congenital heart disease (CHD)-associated aortopathy is a very heterogeneous entity with a wide spectrum of clinical presentations. The pathogenesis of aortopathy is still incompletely understood, and, therefore, the best prevention and management strategy is currently unknown. The most common entity of CHD-associated aortopathies is bicuspid aortic valve (BAV)-associated aortic disease (so called bicuspid aortopathy) that is found in 50%–60% of BAV individuals. BAV aortopathy has been reported in association with an increased risk of aortic events, especially aortic dissection and sudden cardiac death. Risk stratification of adverse aortic events is still very rudimentary and considers only the maximal aortic diameter, which makes it unsuitable for an individual risk prediction. This introductory Editorial highlights the unmet clinical need for more integrative and translational research to unravel pathogenetic pathways in the development of CHD-associated aortopathies, integrating recently identified genetic lesions and knowledge on circulating biomarkers and microstructural changes in the diseased aorta. MDPI 2020-01-11 /pmc/articles/PMC7019613/ /pubmed/31940858 http://dx.doi.org/10.3390/jcm9010204 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 Editorial
Girdauskas, Evaldas
Kaemmerer, Harald
von Kodolitsch, Yskert
Unravelling the Pathogenetic Mechanisms in Congenital Aortopathies: Need for an Integrative Translational Approach
title Unravelling the Pathogenetic Mechanisms in Congenital Aortopathies: Need for an Integrative Translational Approach
title_full Unravelling the Pathogenetic Mechanisms in Congenital Aortopathies: Need for an Integrative Translational Approach
title_fullStr Unravelling the Pathogenetic Mechanisms in Congenital Aortopathies: Need for an Integrative Translational Approach
title_full_unstemmed Unravelling the Pathogenetic Mechanisms in Congenital Aortopathies: Need for an Integrative Translational Approach
title_short Unravelling the Pathogenetic Mechanisms in Congenital Aortopathies: Need for an Integrative Translational Approach
title_sort unravelling the pathogenetic mechanisms in congenital aortopathies: need for an integrative translational approach
topic Editorial
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7019613/
https://www.ncbi.nlm.nih.gov/pubmed/31940858
http://dx.doi.org/10.3390/jcm9010204
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