CAUSES OF PRIMARY PULMONARY HYPERTENSION (PPH)?

No one is certain as to what causes primary pulmonary hypertension (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 immune system disease, congenital heart defects, portal hypertension, HIV infection, Collagen Vascular Disease, and intravenous drug use or other chemical exposures. The use of certain appetite suppressants, such as Pondimin (fenfluramine) and  Redux (dexfenfluramine), have been found to greatly increase the risk of developing PPH. Recent medical research indicates that Herbal Fen-Phen (ephedra / ma huang) may be the cause for PPH in some victims.The natural amphetemines found in the ephedra in the form of ephedrine alkaloids may cause of primary pulmonary hypertension.

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. An echocardiogram is a good tool for detecting elevated pulmonary artery pressures. However, with some patients a cardiac catheterization is necessary to confirm the diagnosis and  gather additional artery pressures.

Patients with Primary Pulmonary Hypertension (PPH) are currently being 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 these medications, others with severe PPH may need a lung transplantation or heart-lung transplantation.