Primary pulmonary hypertension (PPH) is a rare disease that results in the progressive narrowing of the blood vessels of the lungs, which as a result causes high blood pressure in these blood vessels and eventually leads to heart failure.
No one is certain as to what causes PPH, but research has shown that there are a number of factors that may be responsible for the disease. Some of the possible causes include genetic or familial predisposition, immune system disease, or drugs or other chemical exposures. The use of certain appetite suppressants, such as fenfluramine and dexfenfluramine, have been found to increase the risk of developing PPH, especially in those people who have used the drugs for more than three months.
The first symptoms of PPH may be very minor, which sometimes delays the diagnosis. A person with PPH may experience shortness of breath following exertion, excessive fatigue, dizziness, fainting, ankle swelling, bluish lips and skin and chest pain. Even if the disease has progressed, the signs and symptoms may be hard to detect or even confused with other conditions that affect the heart and the lungs. A physician may determine if a patient has PPH through a cardiac catheterization.
PPH is treated with a number of drugs, which can't 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, which reduces 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 with severe PPH may need a lung transplantation or heart-lung transplantation.
It is hard to detect the number of PPH cases, however, it is estimated that there are 300 new cases per year. PPH is more common in women between the ages of 21 and 40, but it can affect anyone at any age.
