The Disease

WHO Group 1 : Pulmonary Arterial Hypertension (PAH)

PAH is a vascular disease of the pulmonary arteries that leads to a progressive narrowing of the blood vessels that provide blood flow from the right side of the heart to the lungs and results in an increase in pulmonary vascular resistance and pulmonary artery pressure.  The elevated pulmonary arterial pressure creates an excessive workload on the right side of the heart and is ultimately associated with heart failure and increased mortality.  Common symptoms of PAH include shortness of breath, reduced exercise capacity, fatigue, weakness, and syncope. There are both sporadic and inherited forms. Despite an improved ability to screen for PAH with echocardiography, it typically presents late in the disease state.

Pulmonary arterial hypertension (PAH) is a rare and progressive disorder characterized by abnormally high blood pressure in the pulmonary artery. It is classified as an orphan disease with an estimated prevalence of 15 to 50 persons per million within the United States.