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Key Points Pulmonary hypertension is a progressive disease of various origins, which has a poor prognosis and affects, in its different forms, more than 100 million people worldwide Pulmonary arterial hypertension (PAH) is now considered to be a vasculopathy in which structural changes driven by excessive vascular cell growth and inflammation have a major role A number of proproliferative signaling pathways involving growth factors, cytokines, metabolic signaling, and elastases and proteases have been identified in the pathophysiology of PAH Clinical studies with tyrosine kinase inhibitors, serotonin antagonists, and soluble guanylate cyclase stimulators are underway in patients with PAH The benefits of progenitor cells for vascular repair in PAH are under active investigation The right ventricular response to increased pressure load is recognized as critical to survival in patients with PAH, and strategies for preserving myocardial function are increasingly attracting interest This is a preview of subscription content, access via your institution Access options Subscribe to this journal Receive 12 print issues and online access $189.00 per year only $15.75 per issue Buy this article Purchase on SpringerLink Instant access to the full article PDF

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