Pulmonary arterial hypertension; Sporadic primary pulmonary hypertension; Familial primary pulmonary hypertension; Idiopathic pulmonary arterial hypertension; Primary pulmonary hypertension; PPH; Secondary pulmonary hypertension
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 aterial hypertension are becoming available. Medicines used to treat pulmonary hypertension include:
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.
People with low oxygen levels in the blood may need oxygen therapy at home.
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:
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.
It is not a good idea to get pregnant if you have this condition.
Call your health care provider if:
Most patients with pulmonary arterial hypertension are treated at centers that specialize in the care of these patients.
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.
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