Alternate Names: Pulmonary hypertension; Idiopathic pulmonary hypertension

Causes and Risks: The cause of primary pulmonary hypertension is unknown. Pulmonary hypertension develops as a response to increased resistance to blood flow. Diffuse narrowing of the pulmonary arterioles occurs. The right side of the heart becomes enlarged due to the increased work load of pumping blood against this resistance. Eventually, progressive heart failure develops.

People at risk are women between 20 and 40 years old, although the disease can affect any sex and age. The incidence is 8 out of 100,000 people. It is rare in children. More common is pulmonary hypertension in infants with persistent fetal circulation.

Prevention: unknown

Symptoms:

• progressive shortness of breath with activity
• hyperventilation
• chest pain under the sternum
• weakness
• fatigue
• fainting
• lightheadedness with upright posture
• dizziness
• coughing up blood

Signs and Tests: Physical examination shows enlargement of the veins in the neck, enlargement of the liver, and edema (swelling due to fluid retention in the tissues).

Tests may include:

• ECG
• chest X-ray
• echocardiogram
• cardiac catheterization
• lung scan
• pulmonary arteriogram

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.

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.

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.