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Primary Pulmonary Hypertension (PPH) is a rare and, as yet, incurable disease. It 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 and may become life threatening. PPH is extremely rare, occurring in about two persons per million population per year.
SYMPTOMS
Typical symptoms of Pulmonary Hypertension can include shortness of breath with minimal exertion, fatigue, chest pain, dizzy spells, swelling in the ankles or legs, bluish lips and skin, racing pulse or palpitations, and fainting.
DIAGNOSIS
Pulmonary Hypertension is rarely discovered in a routine medical examination. PPH can be misdiagnosed and has often progressed to late stage by the time it is accurately detected. In its later stages, the signs of the disease can be confused with other conditions affecting the heart and lungs. To effectively detect PPH, doctors often use more sophisticated means such as an echocardiogram or a catheterization.
Pulmonary Hypertension has been historically chronic and incurable with a poor survival rate. However, new treatments are available which have significantly improved prognosis. Recent data indicate that the length of survival is continuing to improve, with some patients able to manage the disorder for 15 to 20 years or longer.
CAUSES
The exact causes of PPH are not yet known. Some researchers believe the blood vessels are particularly sensitive to certain internal or external factors, and constrict, or narrow, when exposed to these factors. There may be a genetic factor, an immune system factor, or sensitivity to drugs or other chemicals. Potential causes are Fen-Phen (fenfluramine and dexfenfluramine), congenital heart diseases, chronic pulmonary thromboembolism (old blood clots in the pulmonary artery), HIV infection, and liver disease.
DIET DRUGS
Studies have revealed an association between some PPH cases and the diet drugs fenfluramine (Pondimin) and dexfenfluramine (Redux). These appetite suppressants are more commonly know as Fen-Phen. If you have PPH and have taken a diet drug, then you may have legal rights to compensation for pain and suffering and treatment.
IMPORTANT ARTICLES
FDA "fen-phen" information page, including the "U.S. Department of Health and Human Services Interim Recommendations Issued for Patients Exposed to Fenfluramine and Dexfenfluramine."
Appetite-Suppressant Drugs and the Risk of Primary Pulmonary Hypertension." (New England Journal of Medicine)
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