Primary Pulmonary Hypertension (PPH)

In 2000, PPH caused a reported 3,065 deaths. It is a rare disease of unknown cause that is also difficult to detect.

What is PPH?

PPH is a rare disease that results in the progressive narrowing of the lung’s blood vessels that causes high blood pressure in these blood vessels, eventually leading to heart failure.


Causes are still unknown. It is more common in women between the ages of 21 and 40, but it can affect anyone at any age. Possible causes include genetic or familial predisposition, immune system disease, or drugs or other chemical exposures.


The initial symptoms of PPH are very minor which often causes the disease to go undetected for a number of years. Typical symptoms may include shortness of breathe after physical exertion; excessive fatigue; dizziness, fainting and weakness; swollen ankles; bluish lips and skin; and chest pain.


PPH is treated with a number of drugs, however they cannot cure or halt the progression of the disease. Drugs are used to alleviate pain. Medication may be used to dilate the blood vessels or keep blood from clotting. Patients with severe PPH may be eligible for lung or heart-lung transplantation.