Document Detail

Cellular and molecular pathobiology of pulmonary arterial hypertension.
MedLine Citation:
PMID:  15194174     Owner:  NLM     Status:  MEDLINE    
Pulmonary arterial hypertension (PAH) has a multifactorial pathobiology. Vasoconstriction, remodeling of the pulmonary vessel wall, and thrombosis contribute to increased pulmonary vascular resistance in PAH. The process of pulmonary vascular remodeling involves all layers of the vessel wall and is complicated by cellular heterogeneity within each compartment of the pulmonary arterial wall. Indeed, each cell type (endothelial, smooth muscle, and fibroblast), as well as inflammatory cells and platelets, may play a significant role in PAH. Pulmonary vasoconstriction is believed to be an early component of the pulmonary hypertensive process. Excessive vasoconstriction has been related to abnormal function or expression of potassium channels and to endothelial dysfunction. Endothelial dysfunction leads to chronically impaired production of vasodilators such as nitric oxide and prostacyclin along with overexpression of vasoconstrictors such as endothelin (ET)-1. Many of these abnormalities not only elevate vascular tone and promote vascular remodeling but also represent logical pharmacological targets. Recent genetic and pathophysiologic studies have emphasized the relevance of several mediators in this condition, including prostacyclin, nitric oxide, ET-1, angiopoietin-1, serotonin, cytokines, chemokines, and members of the transforming-growth-factor-beta superfamily. Disordered proteolysis of the extracellular matrix is also evident in PAH. Future studies are required to find which if any of these abnormalities initiates PAH and which ones are best targeted to cure the disease.
Marc Humbert; Nicholas W Morrell; Stephen L Archer; Kurt R Stenmark; Margaret R MacLean; Irene M Lang; Brian W Christman; E Kenneth Weir; Oliver Eickelberg; Norbert F Voelkel; Marlene Rabinovitch
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Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  Journal of the American College of Cardiology     Volume:  43     ISSN:  0735-1097     ISO Abbreviation:  J. Am. Coll. Cardiol.     Publication Date:  2004 Jun 
Date Detail:
Created Date:  2004-06-14     Completed Date:  2004-07-27     Revised Date:  2005-11-16    
Medline Journal Info:
Nlm Unique ID:  8301365     Medline TA:  J Am Coll Cardiol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  13S-24S     Citation Subset:  AIM; IM    
Service de Pneumologie et Réanimation Respiratoire, Centre des Maladies Vasculaires Pulmonaires, UPRES EA2705, Hôpital Antoine-Béclère, Université Paris-Sud, Clamart, France.
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MeSH Terms
Endothelium, Vascular / cytology,  metabolism,  physiopathology
Hypertension, Pulmonary / etiology*,  metabolism,  pathology*,  physiopathology
Molecular Biology*
Muscle, Smooth, Vascular / cytology,  metabolism,  physiopathology
Pulmonary Artery / metabolism,  pathology*,  physiopathology
Vascular Resistance / physiology
Vasoconstriction / physiology

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