Idiopathic Pulmonary Arterial Hypertension
(IPAH; Unexplained Pulmonary Hypertension; Idiopathic Pulmonary Hypertension; Sporadic Primary Pulmonary Hypertension; Familial Primary Pulmonary Hypertension)
Pronounced: PRY-mair-ee PUL-mo-nair-ee hi-per-TEN-shun
by Kelly de la Rocha
Idiopathic pulmonary arterial hypertension (IPAH) is a rare disease. It is high blood pressure in the arteries (blood vessels) of the lungs.
The right side of the heart will have to work harder to push blood to the lungs. Over time this can lead to heart failure.
The cause of IPAH is unknown.
A person with IPAH has extra muscle in the walls of these blood vessels. That extra muscle makes it more difficult for blood to flow through them.
IPAH is more common in women aged 50 to 55 years. Other factors that may increase your risk of IPAH include:
Initial symptoms of IPAH may be minor. They will get progressively worse. IPAH may cause:
Your doctor will ask about your symptoms and past health. A physical exam will be done. Diagnosis of IPAH may be delayed. Symptoms are similar to other more common conditions like asthma. It is hard to detect IPAH in early stages.
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 IPAH. Treatment is used to help alleviate and control the symptoms and slow progress of the disease. Talk with your doctor about the best plan for you. Treatment includes the following:
Medicine 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 therapy may be given. It may be given through a mask or tubes inserted into the nostrils.
If IPAH is severe or other treatment methods fail, a lung transplant or heart-lung transplant may be needed.
There are no current guidelines to prevent IPAH because the cause is unknown.
National Heart, Lung, and Blood Institute
Pulmonary Hypertension Association
Heart and Stroke Foundation of Canada
The Lung Association
Explore pulmonary hypertension. National Heart, Lung, and Blood Institute website. Available at: https://www.nhlbi.nih.gov/health/health-topics/topics/pah. Accessed January 29, 2019.
Primary pulmonary hypertension in children. Cincinnati Children’s Hospital website. Available at: https://www.cincinnatichildrens.org/health/p/pulmonary-hypertension. Updated June 2014. Accessed September 14, 2017.
Pulmonary arterial hypertension (PAH). EBSCO DynaMed website. Available at: https://www.dyname.... Updated November 20, 2018. Accessed January 29, 2019.
Pulmonary hypertension. American Lung Association website. Available at:
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Accessed January 29, 2019.
Pulmonary hypertension—high blood pressure in the heart-to-lung system. American Heart Association website. Available at:
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Updated May 23, 2017. Accessed January 29, 2019.
Last reviewed September 2020 by EBSCO Medical Review Board Michael J. Fucci, DO, FACC
Last Updated: 8/14/2020
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