Primary pulmonary hypertension

Definition:
A disorder in which the blood pressure in the arteries of the lung is abnormally high, in the absence of other diseases of the heart or lungs.

Alternative Names:
Pulmonary arterial hypertension; Sporadic primary pulmonary hypertension; Familial primary pulmonary hypertension

Causes, incidence, and risk factors:
The cause of primary pulmonary hypertension is unknown. Some cases are caused by a genetic defect. The small arteries of the lung narrow throughout the lungs. Pulmonary hypertension develops as a response to increased resistance to blood flow. As a result of the increased workload caused by this resistance, the right side of the heart becomes enlarged. Eventually, progressive heart failure may develop.

This disease more commonly affects women than men. It is rare, affecting 1 to 2 out of 1,000,000 people.


Review Date: 12/2/2001
Reviewed By: Steven Kawut, M.D., Pulmonary Hypertension Center, Division of Pulmonary, Allergy, and Critical Care Medicine, Columbia University, College of Physicians and Surgeons, New York City, NY. Review provided by VeriMed Healthcare Network.

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