Primary pulmonary hypertension (PPH) is high blood pressure in the lungs. It is also known as idiopathic pulmonary arterial hypertension. It’s a rare lung disorder in which the blood vessels in the lungs narrow and the pressure in the pulmonary artery rises far above normal levels. The pulmonary arteries carry blood from your body to the lungs where carbon dioxide is traded for oxygen.
Pulmonary hypertension is a serious, chronic disease that can lead to heart failure if it’s not treated.
The cause of primary pulmonary hypertension is unknown. Often, there is no underlying heart or lung disease causing the high blood pressure.
Some forms of pulmonary hypertension are linked to a gene defect that can run in families. Researchers believe this gene mutation makes the blood vessels more sensitive to certain factors and they constrict, or narrow, when exposed to these factors.
The following are the most common symptoms for primary pulmonary hypertension. Symptoms can develop so slowly that you can have it for years without knowing it. However, each person may experience symptoms differently. Symptoms may include:
These symptoms get worse as the disease progresses. More severe symptoms are a sign of more advanced disease. In advanced stages, you may:
The symptoms of primary pulmonary hypertension looks like other conditions or medical problems. This condition can be hard to diagnose. Many people ignore symptoms because they think they are simply out of shape. If you or a family member have any of these symptoms, talk with your healthcare provider for a diagnosis.
Primary pulmonary hypertension is rarely discovered in a routine medical exam. In its later stages, the signs of the disease can be confused with other conditions affecting the heart and lungs.
It may be diagnosed when other diseases are ruled out. Tests may include:
There is no cure for primary pulmonary hypertension. Treatment tries to manage symptoms and may include one or more of the following:
Some people also need supplemental oxygen delivered through nasal prongs or a mask if breathing becomes difficult.
This may be a choice for people with severe disease.
The condition can be made worse by a number of factors. You can do certain things that will help you stay as healthy as possible. To maintain a healthy lifestyle with primary pulmonary hypertension:
Tips to help you get the most from a visit to your healthcare provider: