Signal Transduction in the Development of Pulmonary Arterial Hypertension
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- Simon Malenfant
- Pulmonary Hypertension Research Group of the Institut universitaire de cardiologie et de pneumologie de Quebec Research Center Laval University Quebec City Canada
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- Anne‐Sophie Neyron
- Pulmonary Hypertension Research Group of the Institut universitaire de cardiologie et de pneumologie de Quebec Research Center Laval University Quebec City Canada
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- Roxane Paulin
- Department of Medicine University of Alberta Edmonton Alberta Canada
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- François Potus
- Pulmonary Hypertension Research Group of the Institut universitaire de cardiologie et de pneumologie de Quebec Research Center Laval University Quebec City Canada
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- Jolyane Meloche
- Pulmonary Hypertension Research Group of the Institut universitaire de cardiologie et de pneumologie de Quebec Research Center Laval University Quebec City Canada
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- Steeve Provencher
- Pulmonary Hypertension Research Group of the Institut universitaire de cardiologie et de pneumologie de Quebec Research Center Laval University Quebec City Canada
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- Sébastien Bonnet
- Pulmonary Hypertension Research Group of the Institut universitaire de cardiologie et de pneumologie de Quebec Research Center Laval University Quebec City Canada
説明
<jats:p>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.</jats:p>
収録刊行物
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- Pulmonary Circulation
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Pulmonary Circulation 3 (2), 278-293, 2013-04
Wiley
