Pulmonary arterial hypertension

Alternative names
Primary pulmonary hypertension; Sporadic Primary pulmonary hypertension; Familial Primary pulmonary hypertension

Definition

Pulmonary hypertension is when the blood pressure in the arteries of the lungs is abnormally high. “Primary” means that there are no other diseases of the heart or lungs causing the high blood pressure.

(Pulmonary = lungs; hypertension = high blood pressure)

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 is the result of greater 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 is rare. It affects more women than men.

Symptoms

     
  • shortness of breath during activity  
  • chest pain  
  • weakness  
  • fatigue  
  • fainting  
  • light-headedness during exercise  
  • dizziness  
  • leg swelling

Signs and tests

A physical examination shows enlargement of the veins in the neck, normal lungs, a heart murmur, enlargement of the liver, and swelling due to fluid retention in the tissues.

Tests may include:

     
  • ECG  
  • chest X-ray  
  • echocardiogram  
  • pulmonary function tests  
  • nuclear lung scan  
  • CT scan of the chest  
  • cardiac catheterization  
  • pulmonary 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.

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.

Johns Hopkins patient information

Last revised: December 6, 2012
by Dave R. Roger, M.D.

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