Potential Molecular Pathways Related to Pulmonary Artery Aneurysm Development: Lessons to Learn from the Aorta.
aortic aneurysm
gene
molecular biology
pulmonary arterial hypertension
pulmonary artery aneurysm
Journal
International journal of molecular sciences
ISSN: 1422-0067
Titre abrégé: Int J Mol Sci
Pays: Switzerland
ID NLM: 101092791
Informations de publication
Date de publication:
04 04 2020
04 04 2020
Historique:
received:
17
03
2020
revised:
03
04
2020
accepted:
03
04
2020
entrez:
9
4
2020
pubmed:
9
4
2020
medline:
14
1
2021
Statut:
epublish
Résumé
Pulmonary arterial hypertension (PAH) is a rare disease caused by pulmonary vascular remodeling. Current vasodilator treatments have substantially improved patients' survival. This improved survival has led to the appearance of complications related to conditions previously underdiagnosed or even ignored, such as pulmonary artery aneurysm (PAA). The presence of a dilated pulmonary artery has been shown to be related to an increased risk of sudden cardiac death among PAH patients. This increased risk could be associated to the development of left main coronary artery compression or pulmonary artery dissection. Nevertheless, very little is currently known about the molecular mechanisms related to PAA. Thoracic aortic aneurysm (TAA) is a well-known condition with an increased risk of sudden death caused by acute aortic dissection. TAA may be secondary to chronic exposure to classic cardiovascular risk factors. In addition, a number of genetic variants have been shown to be related to a marked risk of TAA and dissection as part of multisystemic syndromes or isolated familial TAA. The molecular pathways implied in the development of TAA have been widely studied and described. Many of these molecular pathways are involved in the pathogenesis of PAH and could be involved in PAA. This review aims to describe all these common pathways to open new research lines that could help lead to a better understanding of the pathophysiology of PAH and PAA and their clinical implications.
Identifiants
pubmed: 32260370
pii: ijms21072509
doi: 10.3390/ijms21072509
pmc: PMC7177585
pii:
doi:
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Subventions
Organisme : Centro de Investigación Biomédica en Red Enfermedades Cardiovasculares
ID : CB16/11/00502
Pays : International
Organisme : Centro de Investigación Biomédica en Red Enfermedades Cardiovasculares
ID : CB16/11/00358
Pays : International
Organisme : Instituto de Salud Carlos III
ID : PI 18/01233
Pays : International
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