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.

Pulmonary arterial hypertension; Sporadic primary pulmonary hypertension; Familial primary pulmonary hypertension; Idiopathic pulmonary arterial hypertension; Primary pulmonary hypertension; PPH; Secondary pulmonary hypertension

The right side of the heart pumps blood through the lungs, where it can receive oxygen.

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, the right side of the heart may become enlarged. At some point, not enough blood flows to the lungs to pick up oxygen and symptoms begin.

At this point, heart failure that involves the right side of the heart is present. This is called cor pulmonale.

Pumonary hypertension may be caused by:

• Any condition that causes chronic low oxygen levels in the blood
• Autoimmune diseases that damage the lungs, such as scleroderma and rheumatoid arthritis
• Certain birth defects of the heart
• Certain diet medications
• Congestive heart failure
• History of a blood clot in the lung
• HIV infection
• Lung or heart valve disease
• Obstructive sleep apnea

In many cases the cause is unknown, in which case the condition is known as idiopathic pulmonary arterial hypertension (IPAH). IPAH is rare. It affects more women than men.

If it is caused by a known medicine or medical condition, it is called secondary pulmonary hypertension.

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Respiratory system

Primary pulmonary hypertension

Review Date: 04/27/2010
Reviewed By: Allen J. Blaivas, DO, Clinical Assistant Professor of Medicine UMDNJ-NJMS, Attending Physician in the Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Veterans Affairs, VA New Jersey Health Care System, East Orange, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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