Primary pulmonary hypertension

Alternative names:

idiopathic pulmonary hypertension; pulmonary hypertension

Treatment:

There is no known cure. The goal of treatment is control of the symptoms. Some people respond to treatment with vasodilators. Other medications used to relieve symptoms include diuretics and calcium channel blockers. As the disease progresses, oxygen may be needed for shortness of breath. Heart-lung transplantation for suitable candidates with available donors has been successful.

Expectations (prognosis):

The probable outcome is poor, most people develop progressive heart failure leading to death in 2 to 8 years.

Complications:

Primary pulmonary hypertension is a progressive disease, leading to congestive heart failure and respiratory failure.

Calling your health care provider:

Call for an appointment with your health care provider if you begin to develop shortness of breath with activity.

Call your health care provider if shortness of breath worsens, if chest pain develops, or if other symptoms of primary pulmonary hypertension develop.