Pulmonary arterial hypertension (PAH) is a serious, progressive, rare disease involving high blood pressure in the pulmonary arteries, which carry blood from the heart to the lungs.1 The cause of PAH is often unknown, but it can be inherited, caused by certain drugs or toxins, or caused by conditions such as connective tissue disease, HIV infection, liver disease, congenital heart disease, sickle cell disease, schistosomiasis, or sequelae to thromboembolic events.2 The most common symptoms include shortness of breath, chest pain, dizziness or fainting, fatigue, and weakness.3 Untreated, PAH can be debilitating, and often fatal.4,5
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- Pulmonary Hypertension Association. About Pulmonary Hypertension (PH) and Pulmonary Arterial Hypertension (PAH). http://www.ourphlibrary.com/module1-about-pah/what-is-pah. Accessed January 2021.
- American Heart Association. Pulmonary Hypertension – High Blood Pressure in the Heart-to-Lung System. https://www.heart.org/en/health-topics/high-blood-pressure/the-facts-about-high-blood-pressure/pulmonary-hypertension-high-blood-pressure-in-the-heart-to-lung-system. Accessed February 2019.
- Rare Disease Report. Rare Disease Quick Facts – Pulmonary Arterial Hypertension (PAH). https://www.raredr.com/news/rare-disease-quick-facts-pah. Accessed February 2019.
- Klinger JR, Elliott CG, Levine DJ, Bossone E, Duvall L, Fagan K, Frantsve-Hawley J, Kawut SM, Ryan JJ, Rosenzweig EB, Sederstrom N, Steen, VD, Badesch DB. Therapy for Pulmonary Arterial Hypertension in Adults: Update of the CHEST Guideline and Expert Panel Report. Chest. 2019;155(3):565-586.
- Delcroix M, Howard L. Pulmonary arterial hypertension: the burden of disease and impact on quality of life. Eur Respir Rev. 2015;24:621-629.