In many cases the cause is unknown. In this case, the condition is called idiopathic pulmonary arterial hypertension (IPAH). It used to be called primary pulmonary hypertension (PPH).
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 known 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 disorders.
Many new treatment options for pulmonary arterial hypertension (IPAH) and other forms of pulmonary arterial hypertension are becoming available. Medicines used to treat pulmonary hypertension include:
Calcium channel blockers
Prostacyclin or similar medicines
Sildenafil and similar medicines
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 medication. Never stop taking medicines without talking to your doctor.
Some patients are put on blood thinners to reduce the risk of blood clots in leg veins and lung arteries.
As the illness gets worse, you will need to make changes in your home and get more help around the house.
Other important tips to follow:
Avoid heavy physical activities and lifting
Avoid traveling to high altitudes
Keep up-to-date with a yearly flu vaccine and pneumococcal vaccine
If treatment with medicine does not work, a lung or heart-lung transplant may help some people.
The long-term outlook has been poor, but new treatments may lead to better results. Some people with this condition may have heart failure that could lead to death. The outlook depends on the condition that is causing pulmonary hypertension.
It is not a good idea to get pregnant if you have this condition.
Calling your health care provider
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: Saunders Elsevier; 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: Saunders Elsevier; 2011:chap 78.
David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Denis Hadjiliadis, MD, Assistant Professor of Medicine, Division of Pulmonary, Allergy and Critical Care, University of Pennsylvania, Philadelphia, PA. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M. Health Solutions, Ebix, Inc.