Information about Primary Pulmonary Hypertension and other forms of Severe Pulmonary Hypertension


PPH NEWS is an education-based resource site that provides information on the health issues surrounding Primary Pulmonary Hypertension (PPH), including what it is, the symptoms, diagnoses, and treatment options.
This website also provides resource to learn more about your legal rights and remedies if you or someone you know has developed PPH due to the use of diet drugs such as Fen Phen.

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PPH Explained

What is PPH?

Primary pulmonary hypertension, also known as PPH, is a rare disease that has no known cause and results in the progressive narrowing of the blood vessels of the lungs. There are no known cures for PPH, but treatment for the cardiovascular condition is improving. The lungs contain millions of tiny blood vessels called capillaries. These capillaries are extremely small, just wide enough for blood cells to move through. The capillaries are lined with what's known as endothelial cells that prevent blood from leaking out of the vessel. On the outside of the blood vessels are muscle cells that expand and contract as blood moves through from the heart into the lungs.

PPH occurs when the vessels gradually stop functioning as they are supposed to. This happens when the cells within the blood vessels weaken and allow blood to leak. This, in turn, causes the exterior muscle cells to constrict. The constriction gradually worsens, eventually driving up the pulmonary arterial pressure. The exact cause of PPH is unknown. However, a prevalent theory holds that the blood vessels react strongly to certain internal and external factors, causing them to constrict when exposed to these factors. Aside from fen-phen and Redux, the causes of PPH include principally congenital heart defects, portal hypertension, HIV infection, Collagen Vascular Disease, and intravenous drug use.

PPH began surfacing through the 1970s because of the latency period found to exist between drug use and PPH diagnosis of 4-6 years. Currently cases of PPH are still being diagnosed. Often times with PPH diagnosis it not immediate because early PPH symptoms are easily mistaken for other causes. This delay in diagnosis results in the disease progressing to late stages without proper treatment.

Secondary pulmonary hypertension (SPH) is the result of other types of lung disease, abnormal breathing processes or heart disease.

What are Heart Valve Defects?

When these valves are impaired, they can cause blood to be regurgitated into the ventricle and left atrium. The symptoms of heart valve disease may be subtle---existent in the presence of shortness of breath and chest pain. Treatment can include medication, a valvoplasty, or a repair of the damaged heart valves and/or heart valve replacement surgery. This condition often requires long term heart monitoring and medications.

 

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Learn More About PPH Treatments:
» Remodulin
» Flolan
» Tracleer


Patients suffering from Primary Pulmonary Hypertension (PPH) caused by Fen-Phen, Pondimin or Redux are not included in the Nationwide Class Action Settlement Agreement with American Home Products.  PPH patients must file independent lawsuits to make legal claims.  To learn your legal rights, please contact us and speak with a PPH Lawyer for lawsuit information!