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—
Tests may include:
Pulse oximetry to evaluate how much oxygen is in your blood
—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
—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 IPAH
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:
Medication 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 (selixipag)
Calcium channel blockers
If breathing becomes difficult,
may be given. It may be given through a mask or tubes inserted into the nostrils.
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: ...(Click grey area to select URL) Accessed January 29, 2019.
Pulmonary hypertension—high blood pressure in the heart-to-lung system. American Heart Association website. Available at: ...(Click grey area to select URL) Updated May 23, 2017. Accessed January 29, 2019.
Last reviewed September 2018 by
EBSCO Medical Review Board
Michael J. Fucci, DO, FACC Last Updated: 1/29/2019