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|Primary Pulmonary Hypertension (PPH)
Primary pulmonary hypertension (PPH) is a rare disease of unknown cause that results in the progressive narrowing of the blood vessels of the lungs, causing high blood pressure in these blood vessels and eventually leading to heart failure. In 2000, there were 3,065 deaths attributed to PPH. Secondary pulmonary hypertension (SPH) is the result of other types of lung disease, abnormal breathing processes or heart disease.
The true incidence of PPH is unknown. However, it is estimated that there are one to two cases per million or 300 new cases per year. In 2000, there were 163,000 hospital discharges in which one of the diagnoses was PPH. It is more common in women between the ages of 21 and 40; however, it can affect anyone at any age.
Initial symptoms of PPH may be very minor, and diagnosis may be delayed for several years until symptoms worsen. Typical symptoms may include:
shortness of breath following exertion
dizziness, fainting, and weakness
bluish lips and skin
It is difficult to detect PPH in a routine medical examination. Even when the disease has progressed, the signs and symptoms may be confused with other conditions that affect the heart and the lungs. To determine if a patient has pulmonary hypertension, a physician may recommend a cardiac catheterization with, perhaps, angiography. PPH is diagnosed only after several possible causes of pulmonary hypertension are excluded; additional tests are usually needed.
No one knows what causes PPH; however, research into the cause suggests a number of factors that may be responsible for the disease. Possible causes include genetic or familial predisposition, immune system disease, or drugs or other chemical exposures.
Use of certain appetite suppressants has been found to increase the risk of developing PPH, especially use lasting more than three months. Studies estimate that treatment with certain appetite suppressant drugs increases the risk of getting PPH from about one to 28 cases per million person-years (one person-year represents a patient treated for one year). Two drugs associated with PPH, fenfluramine and dexfenfluramine, were taken off the market in September 1997 after being linked to heart valve damage.
The prognosis for patients with PPH can be quite variable. Many patients report that by changing some parts of their lifestyles, they can go about many of their daily tasks. The median period of survival is three years after diagnosis, although the survival rate is generally longer for those patients without heart failure and for those patients diagnosed after 40.
Primary pulmonary hypertension is treated with a number of drugs. None of the drugs cure or halt the progression of this disease, but they may relieve symptoms. Some patients take vasodilators, which help to dilate the blood vessels in the lungs, reducing the blood pressure in them. In addition, anticoagulants may be used to decrease the tendency of the blood to clot in the lungs.
Although some patients do well with medication, others may need and be eligible for transplantation. Patients with severe PPH may be candidates for lung transplantation or heart-lung transplantation.
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