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Primary pulmonary hypertension

• Overview
• Symptoms
• Treatment

Alternative Names:

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

Treatment:

There is no known cure. The goal of treatment is to control symptoms.

Medicines used to treat pulmonary hypertension include:

• Ambrisentan (Letairis)
• Bosentan
• Calcium channel blockers
• Diuretics
• Prostacyclin

Your doctor will decide which medicine is best for you.

Persons with advanced cases of pulmonary hypertension may need oxygen. If treatment with medications fails, suitable candidates may be helped by lung or heart-lung transplant .

Expectations (prognosis):

The outlook has traditionally been poor, but new therapies may produce better results. Some people with this condition may develop progressive heart failure leading to death.

Calling your health care provider:

Call your health care provider if:

• You begin to develop shortness of breath when you are active
• Shortness of breath worsens
• Chest pain develops
• Other symptoms develop

Most patients with primary pulmonary hypertension are treated at centers which specialize in the care of these patients.

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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