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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The cause of PPH is unknown. Several factors may contribute to the development of the disease, including:
Immune system diseaseExposure to certain drugs or chemicalsGenetic defects
The following factors increase your chances of developing PPH:
Female between the ages of 30 and 40Liver cirrhosisPortal hypertensionHIV infectionFamily history of PPHUse of appetite suppressants—diet drugs (eg, fenfluramine)Cocaine use
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.
Shortness of breath, especially when you are active or at restHyperventilation—abnormally rapid, deep breathingFatigue—tirednessProgressive weaknessFainting spellsDizzinessCoughing up bloodCyanosis—a bluish tint to the lips and skinSwelling of the legs and handsChest painLack of appetiteCold hands and feetLow blood pressure
A physical exam by your doctor may show:
Swelling of the veins in your neckEnlarged liver and swollen abdomen
—an abnormal sound in the heart
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:
Blood tests—liver function, coagulation tests, test for HIV, autoantibodyBlood gases to determine the oxygen statusElectrocardiogram (ECG, EKG)
—a test that records the heart’s electrical activity
—a test that uses radiation to take a picture of structures inside the body and can show if the heart is enlarged
CT scan of the chest—a type of x-ray that uses a computer to make pictures of structures inside the chestEchocardiogram
—a test that uses high-frequency sound waves (ultrasound) to examine the size, shape and motion of the heart
Pulmonary function tests—non-invasive tests, like blowing into a tube, that measure how well your lungs are workingCardiac catheterization
—to detect problems with the heart and its blood supply
Pulmonary arteriogram—a type of x-ray to detect blood clots and other blockagesNuclear lung scan—a special camera takes pictures of the lungs, to look for blockages in the blood vesselsSix minute walk to determine the amount of shortness of breath, an indirect measure of the severity of PHH
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 ( Nifedipine , diltiazem , and amlodipine )—to relax the muscles in the blood vessels in the lungsProtacylin drugs ( epoprostenol , treprostinil ) and synthetic prostacylins ( iloprost , cisaprost)—to relax the muscles in the blood vessels in the lungsDigoxin —to improve the ability of the heart to pump bloodAnticoagulants—to decrease the chance of blood clots in the lungsDiuretics—to reduce the amount of fluid in the body and in turn reduce strain on the heartVasodilators—to help reduce blood pressure in the blood vessels in the lungsEndothelin receptor antagonists (Bosentan)—to relax muscles in blood vessels of lungsPhosphodiesterase-5 inhibitors ( sildenafil )—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.
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.
American Heart Association. Primary or unexplained pulmonary hypertension. American Heart Association website. Available at:
. Accessed November 10, 2010.
Cedars-Sinai Medical Center.
Imaging–nuclear lung scan. Cedars-Sinai Medical Center website. Available at:
http://www.csmc.edu/pf_2760.html. Accessed September 21, 2005.
Rich S. the current treatment of pulmonary arterial hypertension: time to redefine success.
Zamanian RT, Haddad F, Doyle RL, Weinacker AB. Management strategies for patients with pulmonary hypertension in the intensive care unit.
Crit Care Med
Last reviewed September 2013 by Michael J. Fucci, DO
Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.
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