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:
Family history of IPAH
Use of appetite suppressants (diet pills)
Initial symptoms of IPAH may be minor. They will get progressively worse. IPAH may cause:
Shortness of breath (when you are active or at rest)
Increased difficulty breathing
Coughing up blood
Bluish tint to the lips and skin—cyanosis
Swelling of the legs and hands
Lack of appetite
Cold hands and feet
Low blood pressure
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:
Swelling of the veins in your neck
Enlarged liver and swollen abdomen
An abnormal sound in the heart—heart murmur
Tests may include:
Pulse oximetry to evaluate how much oxygen is in your blood
ECG—to test your heart’s electrical activity
Pulmonary function tests—noninvasive tests, like blowing into a tube, that measure how well your lungs are working
Cardiac catheterization—to detect problems with the heart and its blood supply
6-minute walk to determine the amount of shortness of breath, an indirect measure of the severity of IPAH
Imaging tests evaluate the lungs and surrounding structures. These may include:
of the chest
Nuclear lung scan
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:
Endothelin receptor blockers
Prostanoids like epoprostenol or treprostinil
Stimulators of soluble guanylate cyclase (riociguat)
Prostacyclin receptor antagonist (selexipag)
Calcium channel blockers
If breathing becomes difficult,
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
may be needed.
There are no current guidelines to prevent IPAH because the cause is unknown.
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 hypertension. American Lung Association website. Available at: http://www.lung.org/lung-health-and-diseases/lung-disease-lookup/pulmonary-hypertension. Accessed January 29, 2019.
Pulmonary hypertension—high blood pressure in the heart-to-lung system. American Heart Association website. Available at: http://www.heart.org/HEARTORG/Conditions/HighBloodPressure/AboutHighBloodPressure/What-is-Pulmonary-Hypertension_UCM_301792_Article.jsp#.Wbr2xrKGNxA. Updated May 23, 2017. Accessed January 29, 2019.
Last reviewed September 2018 by
EBSCO Medical Review Board
Michael J. Fucci, DO, FACC
Last Updated: 7/29/2020
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