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The Biological Bases of Group 2 Pulmonary Hypertension

Pulmonary hypertension (PH) is a potentially fatal condition with a prevalence of around 1% in the world population and most commonly caused by left heart disease (PH-LHD). Usually, in PH-LHD, the increase of pulmonary pressure is only conditioned by the retrograde transmission of the left atrial pr...

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Autores principales: Fernández, Ana I., Yotti, Raquel, González-Mansilla, Ana, Mombiela, Teresa, Gutiérrez-Ibanes, Enrique, Pérez del Villar, Candelas, Navas-Tejedor, Paula, Chazo, Christian, Martínez-Legazpi, Pablo, Fernández-Avilés, Francisco, Bermejo, Javier
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6928720/
https://www.ncbi.nlm.nih.gov/pubmed/31771195
http://dx.doi.org/10.3390/ijms20235884
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author Fernández, Ana I.
Yotti, Raquel
González-Mansilla, Ana
Mombiela, Teresa
Gutiérrez-Ibanes, Enrique
Pérez del Villar, Candelas
Navas-Tejedor, Paula
Chazo, Christian
Martínez-Legazpi, Pablo
Fernández-Avilés, Francisco
Bermejo, Javier
author_facet Fernández, Ana I.
Yotti, Raquel
González-Mansilla, Ana
Mombiela, Teresa
Gutiérrez-Ibanes, Enrique
Pérez del Villar, Candelas
Navas-Tejedor, Paula
Chazo, Christian
Martínez-Legazpi, Pablo
Fernández-Avilés, Francisco
Bermejo, Javier
author_sort Fernández, Ana I.
collection PubMed
description Pulmonary hypertension (PH) is a potentially fatal condition with a prevalence of around 1% in the world population and most commonly caused by left heart disease (PH-LHD). Usually, in PH-LHD, the increase of pulmonary pressure is only conditioned by the retrograde transmission of the left atrial pressure. However, in some cases, the long-term retrograde pressure overload may trigger complex and irreversible biomechanical and biological changes in the pulmonary vasculature. This latter clinical entity, designated as combined pre- and post-capillary PH, is associated with very poor outcomes. The underlying mechanisms of this progression are poorly understood, and most of the current knowledge comes from the field of Group 1-PAH. Treatment is also an unsolved issue in patients with PH-LHD. Targeting the molecular pathways that regulate pulmonary hemodynamics and vascular remodeling has provided excellent results in other forms of PH but has a neutral or detrimental result in patients with PH-LHD. Therefore, a deep and comprehensive biological characterization of PH-LHD is essential to improve the diagnostic and prognostic evaluation of patients and, eventually, identify new therapeutic targets. Ongoing research is aimed at identify candidate genes, variants, non-coding RNAs, and other biomarkers with potential diagnostic and therapeutic implications. In this review, we discuss the state-of-the-art cellular, molecular, genetic, and epigenetic mechanisms potentially involved in PH-LHD. Signaling and effective pathways are particularly emphasized, as well as the current knowledge on -omic biomarkers. Our final aim is to provide readers with the biological foundations on which to ground both clinical and pre-clinical research in the field of PH-LHD.
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spelling pubmed-69287202019-12-26 The Biological Bases of Group 2 Pulmonary Hypertension Fernández, Ana I. Yotti, Raquel González-Mansilla, Ana Mombiela, Teresa Gutiérrez-Ibanes, Enrique Pérez del Villar, Candelas Navas-Tejedor, Paula Chazo, Christian Martínez-Legazpi, Pablo Fernández-Avilés, Francisco Bermejo, Javier Int J Mol Sci Review Pulmonary hypertension (PH) is a potentially fatal condition with a prevalence of around 1% in the world population and most commonly caused by left heart disease (PH-LHD). Usually, in PH-LHD, the increase of pulmonary pressure is only conditioned by the retrograde transmission of the left atrial pressure. However, in some cases, the long-term retrograde pressure overload may trigger complex and irreversible biomechanical and biological changes in the pulmonary vasculature. This latter clinical entity, designated as combined pre- and post-capillary PH, is associated with very poor outcomes. The underlying mechanisms of this progression are poorly understood, and most of the current knowledge comes from the field of Group 1-PAH. Treatment is also an unsolved issue in patients with PH-LHD. Targeting the molecular pathways that regulate pulmonary hemodynamics and vascular remodeling has provided excellent results in other forms of PH but has a neutral or detrimental result in patients with PH-LHD. Therefore, a deep and comprehensive biological characterization of PH-LHD is essential to improve the diagnostic and prognostic evaluation of patients and, eventually, identify new therapeutic targets. Ongoing research is aimed at identify candidate genes, variants, non-coding RNAs, and other biomarkers with potential diagnostic and therapeutic implications. In this review, we discuss the state-of-the-art cellular, molecular, genetic, and epigenetic mechanisms potentially involved in PH-LHD. Signaling and effective pathways are particularly emphasized, as well as the current knowledge on -omic biomarkers. Our final aim is to provide readers with the biological foundations on which to ground both clinical and pre-clinical research in the field of PH-LHD. MDPI 2019-11-23 /pmc/articles/PMC6928720/ /pubmed/31771195 http://dx.doi.org/10.3390/ijms20235884 Text en © 2019 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
Fernández, Ana I.
Yotti, Raquel
González-Mansilla, Ana
Mombiela, Teresa
Gutiérrez-Ibanes, Enrique
Pérez del Villar, Candelas
Navas-Tejedor, Paula
Chazo, Christian
Martínez-Legazpi, Pablo
Fernández-Avilés, Francisco
Bermejo, Javier
The Biological Bases of Group 2 Pulmonary Hypertension
title The Biological Bases of Group 2 Pulmonary Hypertension
title_full The Biological Bases of Group 2 Pulmonary Hypertension
title_fullStr The Biological Bases of Group 2 Pulmonary Hypertension
title_full_unstemmed The Biological Bases of Group 2 Pulmonary Hypertension
title_short The Biological Bases of Group 2 Pulmonary Hypertension
title_sort biological bases of group 2 pulmonary hypertension
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6928720/
https://www.ncbi.nlm.nih.gov/pubmed/31771195
http://dx.doi.org/10.3390/ijms20235884
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