title: Signal transduction in the development of pulmonary arterial hypertension.
abstract: Pulmonary arterial hypertension (PAH) is a unique disease. Properly speaking, it is not a disease of the lung. It can be seen more as a microvascular disease occurring mainly in the lungs and affecting the heart. At the cellular level, the PAH paradigm is characterized by inflammation, vascular tone imbalance, pulmonary arterial smooth muscle cell proliferation and resistance to apoptosis and the presence of in situ thrombosis. At a clinical level, the aforementioned abnormal vascular properties alter physically the pulmonary circulation and ventilation, which greatly influence the right ventricle function as it highly correlates with disease severity. Consequently, right heart failure remains the principal cause of death within this cohort of patients. While current treatment modestly improve patients' conditions, none of them are curative and, as of today, new therapies are lacking. However, the future holds potential new therapies that might have positive influence on the quality of life of the patient. This article will first review the clinical presentation of the disease and the different molecular pathways implicated in the pathobiology of PAH. The second part will review tomorrow's future putative therapies for PAH.
authors.affiliation: Pulmonary Hypertension Research Group of the Institut universitaire de cardiologie et de pneumologie de Quebec Research Center, Laval University, Quebec City, Canada.
authors.forename: Simon
authors.initials: S
authors.lastname: Malenfant
authors.forename: Anne-Sophie
authors.initials: AS
authors.lastname: Neyron
authors.forename: Roxane
authors.initials: R
authors.lastname: Paulin
authors.forename: François
authors.initials: F
authors.lastname: Potus
authors.forename: Jolyane
authors.initials: J
authors.lastname: Meloche
authors.forename: Steeve
authors.initials: S
authors.lastname: Provencher
authors.forename: Sébastien
authors.initials: S
authors.lastname: Bonnet
keywords: future therapies; micro-ribonucleic acid; pulmonary arterial hypertension; remodeling pathways; vascular tone imbalance