Pulmonary hypertension is a rare blood vessel disorder of the lung in which the pressure in the pulmonary artery (the blood vessel that leads from the heart to the lungs) rises above normal levels. This may become life-threatening.
Symptoms of pulmonary hypertension include:
When this occurs in the absence of a known cause, it is referred to as idiopathic pulmonary hypertension (IPH). There are likely many unknown causes of IPH. It is extremely rare, occurring in about two in one million people.
There are other causes of pulmonary hypertension. These include breathing disorders such as emphysema, scleroderma, CREST syndrome or systemic lupus erythematosus (SLE). Congenital heart diseases may also be a cause, as can HIV infection, liver disease and diet drugs such as fenfluramine and dexfenfluramine.
Pulmonary hypertension is frequently misdiagnosed and often progresses to late stages by the time it is accurately diagnosed. This is because pulmonary hypertension is a progressive, incurable and possibly fatal disease that needs to be diagnosed and treated quickly to improve prognosis and survival rate.
With early and correct treatment, the length of survival is continuing to improve, with some patients able to manage the disorder for 15 to 20 years or longer.
For more information about the Pulmonary Hypertension Clinic at Rhode Island Hospital and its therapy options, or to schedule an appointment, please call 401-444-3570.
Learn more about pulmonary hypertension in our Health Encyclopedia.