Pulmonary arterial hypertension (PAH) is a chronic, life-threatening disorder which is characterized by abnormally high blood pressure in the pulmonary artery. The pulmonary artery carries blood from the heart to the lungs in order to provide fresh oxygen throughout the body. If PAH is left untreated, the walls of the pulmonary artery and surrounding vasculature can harden and thicken, causing disruption of blood flow between the heart and lungs. Progressive symptoms of PAH include shortness of breath on exertion, chest pain, fainting, fluid retention, and death due to right ventricular failure.

In many cases, no specific cause of PAH can be identified. Known causes of PAH include certain forms of congenital heart disease that cause excessive pulmonary blood flow, lung disease, chronic pulmonary thromboembolism, HIV infection, liver disease, and diet drugs like fenfluramine and dexfenfluramine. PAH can also be caused by inflammatory or collagen vascular diseases such as scleroderma, CREST syndrome, or systemic lupus erythematosus (SLE). In some cases PAH can be hereditary.

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