The Pulmonary Hypertension Academic Research Consortium

The search for effective treatments for pulmonary hypertension (PH) has been frustrating. The very first drug for idiopathic pulmonary arterial hypertension (PAH), epoprostenol, was approved in 1995.[1] And while progress has been made, a look at where we were, where we are, and where we need to be suggests we are far from accomplishing our goals.[2] We have identified three classes of drugs that improve symptoms, but we still do not have drugs that modify the disease process or protect patients from developing progressive pulmonary vascular disease.[3] Perhaps this is because the developed drugs were studied due to their vasodilator properties, while scientific research has now demonstrated that cellular proliferation, inflammation, and thrombosis are the dominant underlying pathobiologic processes, with chronic pulmonary vasoconstriction playing a relatively minor role.[4]