Treatment options in pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) is a rare, severe and progressive disease characterised by raised pressures in the arteries in the lungs leading to right heart failure and, historically, a median life expectancy of 2.8 years with-out targeted treatment.¹ Pathologically, it is characterised by a thickening of the muscular layer of the arteries, dysfunction of the vessel walls, and abnormal growth of supporting cells such as fibroblasts. Copyright © 2007 Wiley Interface Ltd