Primary pulmonary hypertension

Disease	Injury	Nutrition	Poison

Special Topic	Surgery	Symptoms	Test

Respiratory system

Primary pulmonary hypertension

Alternative Names:

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

Treatment:

There is no known cure. The goal of treatment is to control symptoms. Some people respond to treatment with oral medications, such as calcium channel blockers and diuretics. Others may need drugs like intravenous epoprostenol.

Recently, a new oral drug that blocks abnormal constriction of blood vessels has become available and shows promise in treating pulmonary hypertension.

As the disease progresses, oxygen may be necessary. If all else fails, suitable candidates may be helped by lung or heart-lung transplantation.

Support Groups:

Pulmonary Hypertension Association

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.

Review Date: 5/14/2003
Reviewed By: David A. Kaufman, M.D., Pulmonary & Critical Care Medicine, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network.

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