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:
Autoimmune diseasesExposure to certain drugs or chemicalsGenetic defects
PPH is more common in women aged 30-40 years. Other factors that may increase your risk of PPH include: Liver cirrhosisPortal hypertensionHIV infectionFamily history of PPHUse of appetite suppressants (diet pills)Cocaine use
Initial symptoms of PPH may be minor. They will get progressively worse. PPH may cause: Shortness of breath (when you are active or at rest)Abnormally rapid, deep breathing—hyperventilationFatigueProgressive weaknessFainting spellsLightheadednessCoughing up bloodBluish tint to the lips and skin—cyanosisSwelling of the legs and handsChest painLack of appetiteCold hands and feetLow blood pressure
Your doctor will ask about your symptoms and medical history. A physical exam will be done. Diagnosis of PPH may be delayed. It is hard to detect until symptoms worsen.
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—heart murmur
Tests may include:
Blood testsPulse oximetry to evaluate how much oxygen is in your bloodElectrocardiogram (EKG)—to test your heart’s electrical activity
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
Six minute walk to determine the amount of shortness of breath, an indirect measure of the severity of PHH
Imaging tests evaluate the lungs and surrounding structures. These may include: Chest x-rayCT scan of the chestEchocardiogramPulmonary arteriogramNuclear lung scan
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:
Medication can improve blood flow, decrease the risk of blood clots, and improve the ability of the heart to pump blood. These may include: Calcium channel blockersProtacylinsDigoxinAnticoagulantsDiureticsVasodilatorsEndothelin receptor antagonistsPhosphodiesterase-5 inhibitors
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 or when other treatment methods fail.
There are no current guidelines for preventing PPH because the cause is not known.
Nuclear lung scan. Cedars-Sinai Medical Center website. Available at:
http://www.cedars-sinai.edu/Patients/Programs-and-Services/Imaging-Center/For-Patients/Exams-by-Procedure/Nuclear-Medicine/Nuclear-Lung-Scan.aspx. Accessed September 25, 2014.
Primary pulmonary hypertension. American Lung Association website. Available at:
http://www.lung.org/lung-disease/primary-pulmonary-hypertension. Accessed September 25, 2014.
Primary pulmonary hypertension in children. Cincinnati Children’s Hospital website. Available at:
http://www.cincinnatichildrens.org/health/p/pulmonary-hypertension. Updated June 2014. Accessed September 25, 2014.
Pulmonary arterial hypertension (PAH). EBSCO DynaMed website. Available at:
http://www.ebscohost.com/dynamed. Updated June 30, 2014. Accessed September 25, 2014.
Rich S. The current treatment of pulmonary arterial hypertension: time to redefine success.
What is pulmonary hypertension? American Heart Association website. Available at:
http://www.heart.org/HEARTORG/Conditions/HighBloodPressure/AboutHighBloodPressure/What-is-Pulmonary-Hypertension_UCM_301792_Article.jsp. Updated August 12, 2014. Accessed September 25, 2014.
Zamanian RT, Haddad F, et al. Management strategies for patients with pulmonary hypertension in the intensive care unit.
Crit Care Med. 2007;35:2037-2050.
Last reviewed August 2014 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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