A disorder in which the blood pressure in the arteries of the lung is abnormally high, in the absence of other diseases of the heart or lungs.
Alternative Names
Pulmonary arterial hypertension; Sporadic primary pulmonary hypertension; Familial primary pulmonary hypertension
Causes, incidence, and risk factors
The cause of primary pulmonary hypertension is unknown. Some cases are caused by a genetic defect. The small arteries of the lung narrow throughout the lungs. Pulmonary hypertension develops as a response to increased resistance to blood flow. As a result of the increased workload caused by this resistance, the right side of the heart becomes enlarged. Eventually, progressive heart failure may develop. This disease more commonly affects women than men. It is rare, affecting 1 to 2 out of 1,000,000 people.
Signs and tests
Physical examination shows enlargement of the veins in the neck, normal lungs, a heart murmur, enlargement of the liver, and edema ( swelling due to fluid retention in the tissues). Tests may include:
ECGchest X-rayechocardiogram pulmonary function tests nuclear lung scan
CT scan of the chest cardiac catheterizationpulmonary arteriogram
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. 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)
Studies show poor outcomes, but these were conducted before new therapies became available. 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. Call your health care provider if shortness of breath worsens, if chest pain develops, or if other symptoms develop. Most patients with primary pulmonary hypertension are treated at centers which specialize in the care of these patients.