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Monsour Law Firm
404 N Green St
Longview, Texas 75601
PO Box 4209
Longview, TX 75606
903-758-5757
1-800-815-2911
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Primary or unexplained pulmonary hypertension (PPH) 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 PPH cannot be diagnosed until all types of secondary pulmonary hypertension (of which there are many) have been excluded on clinical grounds, 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 PPH.
A significant association exists between the use of the fen-phen diet drug and PPH. Fen-phen was taken off the market in the U.S. in 1997. Studies have shown that patients can develop the disease several years after they stopped taking diet drugs. Medical experts have testified that there is a potential latency of ten or more years between the last date on which a patient is exposed to diet drugs and the date at which the patient develops the first symptoms of what is ultimately diagnosed as PPH. If you have PPH and took fen-phen, please contact us.
The most common symptoms of primary pulmonary hypertension are related to difficulty with breathing. They include:
These symptoms may occur at presentation, or progress over a period of time. A patient with primary pulmonary hypertension must be monitored for these symptoms by routine visits to their cardiologist or pulmonologist. If any new symptoms develop, patients should notify their doctor immediately.
A physical examination of patients with primary pulmonary hypertension may show:
All of these signs are related to the right ventricle working harder to pump blood into the high resistance of the lungs' blood vessels.
There may be one or more causes of PPH, however, all remain unknown. The low incidence makes learning more about the disease extremely difficult. Studies of PPH also have been difficult because a good animal model of the disease has not been available. Nevertheless, researchers think that in most people who develop PPH the blood vessels are particularly sensitive to certain internal or external factors and constrict, or narrow, when exposed to these factors.
Diet Drugs - Fen-Phen
A significant association exists between the use of the fen-phen diet drug and PPH. Fen-phen was taken off the market in the U.S. in 1997. Studies have shown that patients can develop the disease several years after having stopped taking diet drugs. Medical experts have testified that there is a potential latency of ten or more years between the last date on which a patient is exposed to diet drugs and the date at which the patient develops the first symptoms of what is ultimately diagnosed as PPH. If you have PPH and took fen-phen, please contact us.
Other Causes of PPH
People with Raynaud's disease seem more likely than others to develop PPH; Raynaud's disease is a condition in which the fingers and toes turn blue when cold because the blood vessels in the fingers and toes are particularly sensitive to cold.
Cocaine, HIV, and pregnancy are some of the factors that are thought to trigger constriction, or narrowing, in the pulmonary artery resulting in PPH. In about 6 to 10 percent of cases, PPH is familial; that is, it is inherited from other family members. The familial form of PPH is similar to the more common form of the disease, sometimes referred to as "sporadic" PPH.