Primary pulmonary hypertension

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Primary pulmonary hypertension

Respiratory system

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

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.

Pulmonary Hypertension Association

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.

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: 02/01/2005
Reviewed By: Allen J. Blaivas, D.O., Division of Pulmonary and Critical Care Medicine, UMDNJ-New Jersey Medical School, Newark, NJ. Review provided by VeriMed Healthcare Network.