In many patients, the cause of pulmonary hypertension is unknown. In this case, the condition is called idiopathic pulmonary arterial hypertension (IPAH). Idiopathic means the cause of a disease is not known. IPAH is rare. It affects more women than men.
If pulmonary hypertension is caused by a known medicine or medical condition, it is called secondary pulmonary hypertension.
Shortness of breath or light-headedness during activity is often the first symptom. Fast heart rate (palpitations) may be present. Over time, symptoms occur with lighter activity or even while at rest.
In the early stages of the disease, the exam may be normal or almost normal. The condition may take several months to diagnose. Asthma and other diseases may cause similar symptoms and must be ruled out.
There is no cure for pulmonary hypertension. The goal of treatment is to control symptoms and prevent more lung damage. It is important to treat medical disorders that cause pulmonary hypertension, such as obstructive sleep apnea, lung conditions, and heart valve problems.
Many treatment options for pulmonary arterial hypertension are available. If you are prescribed medicines, they may be taken by mouth (oral), received through the vein (intravenous, or IV), or breathed in (inhaled).
Your doctor will decide which medicine is best for you. You will be closely monitored during treatment to watch for side effects and to see how well you are responding to the medicine. Do not stop taking your medicines without talking to your doctor.
Other treatments may include:
Blood thinners to reduce the risk of blood clots, especially if you have IPAH
Get a yearly flu vaccine, as well as other vaccines such as the pneumonia vaccine
How well you do depends on what caused the condition.
As the illness gets worse, you will need to make changes in your home to help you get around the house.
When to Contact a Medical Professional
Call your health care provider if:
You begin to develop shortness of breath when you are active
Shortness of breath gets worse
You develop chest pain
You develop other symptoms
Most patients with pulmonary arterial hypertension are treated at centers that specialize in the care of this condition.
Channick RN, Rubin LJ. Pulmonary hypertension. In: Mason RJ, Broaddus VC, Martin TR, et al., eds. Murray and Nadel's Textbook of Respiratory Medicine. 5th ed. Philadelphia, Pa.: Elsevier Saunders; 2010:chap 52.
McLaughlin VV, Archer SL, Badesch DB, Barst RJ, Farber HW, Lindner JR, et al: American College of Cardiology Foundation Task Force on Expert Consensus Documents; American Heart Association; American College of Chest Physicians; American Thoracic Society, Inc; Pulmonary Hypertension Association. ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association developed in collaboration with the American College of Chest Physicians: American Thoracic Society, Inc; and the Pulmonary Hypertension Association. J Am Coll Cardiol. 2009;53:1573-1619.
Rich S. Pulmonary hypertension. In: Bonow ROL, Mann DL, Zipes DP, Libby P, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 9th ed. Philadelphia, Pa.: Elsevier Saunders; 2011:chap 78.
Denis Hadjiliadis, MD, Assistant Professor of Medicine, Division of Pulmonary, Allergy and Critical Care, University of Pennsylvania, Philadelphia, PA. Also reviewed by A.D.A.M. Health Solutions, Ebix, Inc., Editorial Team: David Zieve, MD, MHA, Bethanne Black, Stephanie Slon, and Nissi Wang.