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.
Heart and Lungs
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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:
Calcium channel-blocking drugs (
)—to relax the muscles in the blood vessels in the lungs
Protacylin drugs (
) and synthetic prostacylins (
, cisaprost)—to relax the muscles in the blood vessels in the lungs
—to improve the ability of the heart to pump blood
- Anticoagulants—to decrease the chance of blood clots in the lungs
- Diuretics—to reduce the amount of fluid in the body and in turn reduce strain on the heart
- Vasodilators—to help reduce blood pressure in the blood vessels in the lungs
- Endothelin receptor antagonists (Bosentan)—to relax muscles in blood vessels of lungs
Phosphodiesterase-5 inhibitors (
)—to relax muscles in blood vessels of lungs
If breathing becomes difficult oxygen may be given. It may be given through a mask or tubes inserted into the nostrils.
Lung Transplant or Heart-Lung Transplant
Defective lungs and/or heart are replaced with donor organs. This option is used only in severe cases of PPH.