Pulmonary arterial hypertension; Sporadic primary pulmonary hypertension; Familial primary pulmonary hypertension; Idiopathic pulmonary arterial hypertension; Primary pulmonary hypertension; PPH; Secondary pulmonary hypertension
Pulmonary hypertension is abnormally high blood pressure in the arteries of the lungs. It makes the right side of the heart need to work harder than normal.
The right side of the heart pumps blood through the lungs, where it picks up oxygen. Then, the blood returns to the left side of the heart, where it is pumped to the whole body.
When the small arteries (blood vessels) of the lung become narrowed, they cannot carry as much blood. When this happens, pressure builds up. This is called pulmonary hypertension.
The heart needs to work harder to force the blood through the vessels against this pressure. Over time, this causes the right side of the heart to become larger. Not enough blood flows to the lungs to pick up oxygen.
At this point, heart failure involves the right side of the heart. This is called cor pulmonale.
Pulmonary hypertension may be caused by:
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.
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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