Primary Arterial Hypertension (PAH) /
Primary Pulmonary Hypertension (PPH)
This website was created to help Pulmonary Arterial Hypertension (PAH), which is also known as Primary Pulmonary Pypertension (PPH), patients, their families, and friends with up-to-date information about PAH and PPH. We have the latest information on PAH/PPH, its symptoms, causes, diagnosis, and treatment. We have also gathered vital information on coping with the personal and financial effects of this rare disease, which may be difficult to deal with without the proper preparation.
Pulmonary Arterial Hypertension(PAH) is now the name for primary or unexplained pulmonary hypertension (PPH). It is a rare lung disorder in which the blood pressure in the pulmonary artery rises far above normal levels for no apparent reason. The pulmonary artery is a blood vessel carrying oxygen-poor blood from the right ventricle (one of the heart's pumping chambers) to the lungs. In the lungs, the blood picks up oxygen, then flows to the heart's left side, where the left ventricle pumps it to the rest of the body through the aorta.
In contrast, secondary pulmonary hypertension (SPH) means the cause is known. A common cause of SPH are the breathing disorders emphysema and bronchitis. Other less frequent causes are the inflammatory or collagen vascular diseases such as scleroderma, CREST syndrome or systemic lupus erythematosus.
Because PAH/PPH cannot be diagnosed until all types of secondary pulmonary hypertension (of which there are many) have been excluded on clinical grounds, PAH/PPH is very difficult to diagnose. In the United States, an estimated 500 to 1,000 new cases of primary pulmonary hypertension are diagnosed each year. The greatest number is reported in women between ages 20 and 40. However, men and women in all age ranges as well as very young children can develop PAH/PPH.
As PAH/PPH is serious, it is very important to seek the right medical treatment as soon as possible. The Treatment Options section describes the different types of treatments, new drugs, PAH/PPH Clinics, and PAH/PPC Medical Doctors. In our Financial Aid section, we discuss how patients and their families may be helped with medical bills and financial concerns.
Because PPH or PAH may be linked to the diet drug fen phen, see our Causes section to find out about this risk factor.
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