Primary pulmonary hypertension (PPH) is a rare disease. It is high blood pressure
in the blood vessels of the lungs.
A person with PPH has extra muscle in the walls of these blood vessels. That extra muscle makes it more difficult for blood to flow through them. As a result, the right side of the heart has to work harder to push blood to the lungs. This additional strain can eventually lead to heart failure.
PPH is a serious condition. It requires care from your doctor.
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The cause of PPH is unknown. Several factors may contribute to the development of the disease, including:
The following factors increase your chances of developing PPH:
Initial symptoms of PPH may be minor. They will get progressively worse. If you experience any of these symptoms do not assume it is due to PPH. These symptoms may be caused by other, less serious health conditions. If you experience any one of them, see your doctor.
A physical exam by your doctor may show:
Diagnosis of PPH may be delayed. It is hard to detect until symptoms worsen. Evaluation should take place at a medical center with expertise in PPH.
Your doctor will ask about your symptoms and medical history. A physical exam will be done. Tests may include the following:
There is no cure for PPH. Treatment is used to help alleviate and control the symptoms. Talk with your doctor about the best plan for you. Treatment options include the following:
If breathing becomes difficult oxygen may be given. It may be given through a mask or tubes inserted into the nostrils.
Defective lungs and/or heart are replaced with donor organs. This option is used only in severe cases of PPH.
There are no guidelines for preventing PPH because the cause is not known.