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Primary pulmonary hypertension

• Overview
• Symptoms
• Treatment

Definition:

Primary pulmonary hypertension is abnormally high blood pressure in the arteries of the lungs.

Alternative Names:

Pulmonary arterial hypertension; Sporadic primary pulmonary hypertension; Familial primary pulmonary hypertension; Idiopathic pulmonary arterial hypertension

Causes, incidence, and risk factors:

The cause of primary pulmonary hypertension is unknown. When the cause is unknown, it is called idiopathic. Some cases are caused by a genetic defect, which may run in families.

In this condition, the small arteries of the lung become narrow, which makes it hard for blood to flow. Blood pressure increases. The right side of the heart must work harder to pump blood, and may eventually become enlarged. Eventually, progressive heart failure may develop.

Similar forms of pulmonary hypertension can develop in persons who take certain diet medicines, or who have severe lung disease, long-standing heart valve disease, or history of a chronic pulmonary embolism.

This disease is rare. It affects more women than men.

References:

McGoon M, Gutterman D, Steen V, et al. Screening, early detection, and diagnosis of pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest . 2004 Jul;126(1 Suppl):14S-34S.

Sahara M, Takahashi T, Imai Y, et al. New insights in the treatment strategy for pulmonary arterial hypertension. Cardiovasc Drugs Ther . 2006 Oct;20(5):377-86.

Hayes D Jr. Idiopathic pulmonary arterial hypertension misdiagnosed as asthma. J Asthma . 2007 Jan-Feb;44(1):19-22.

Austin ED, Loyd JE. Genetics and mediators in pulmonary arterial hypertension. Clin Chest Med . 2007 Mar;28(1):43-57.

• Review Date: 6/15/2007
• Reviewed By: Updated by: A.D.A.M. Editorial Team: Greg Juhn, M.T.P.W., David R. Eltz, Kelli A. Stacy. Previously reviewed by David A. Kaufman, M.D., Assistant Professor, Division of Pulmonary, Critical Care & Sleep Medicine, Mount Sinai School of Medicine, New York, NY. Review provided by VeriMed Healthcare Network. (March 2007)

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