Kelly de la Rocha
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.
The cause of PPH is unknown. Several factors may contribute to the development of the disease, including:
PPH is more common in women aged 30-40 years. Other factors that may increase your risk of PPH include:
Initial symptoms of PPH may be minor. They will get progressively worse. PPH may cause:
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:
Tests may include:
Imaging tests evaluate the lungs and surrounding structures. These may include:
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:
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.
PPH Cure Foundation
Pulmonary Hypertension Association
Canadian Lung Association
Heart and Stroke Foundation of Canada
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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