Patient Information: Adult Illnesses

What You Should Know About

Primary Pulmonary Hypertension

Primary pulmonary hypertension (PPH) is a rare but serious disease that causes progressive narrowing of blood vessels in the lungs. As a result, the blood pressure in the lungs rises and eventually leads to heart failure. Pulmonary hypertension also can result from other lung diseases. When that happens, the resulting disorder is known as secondary pulmonary hypertension.

No one knows what causes PPH; however, researchers have suggested that it may run in families or it may be related to immune system disease or exposure to certain drugs or chemicals.

PPH can affect anyone of any age, but it's most common among women ages 21 to 40. It typically starts with very mild shortness if breath that progresses slowly. It may be several years before the symptoms are bothersome enough to see a doctor. Typical symptoms may include:

• shortness of breath following exertion
• excessive fatigue
• dizziness, fainting, and weakness
• ankle swelling
• bluish lips and skin
• chest pain.
  

Diagnosing PPH is difficult in a routine medical examination because the symptoms are similar to many other disorders that affect the lungs and heart. To reach a firm diagnosis it may be necessary to have a heart cathaterization.. This procedure allows doctors to examine the blood vessels in your heart and lungs, and determine the blood pressure in the lungs.

Treatment and Tips

PPH can't be cured, but your doctor may prescribe several drugs to reduce the symptoms. For example, you may take a vasodilator to dilate blood vessels in the lungs, thus reducing pressure inside them. You also may take an anticoagulant to decrease the tendency of blood to clot in the lungs.

When the symptoms of PPH become severe despite medication, you may be eligible for lung or heart-lung transplantation. In the meantime, you may find it possible to pursue many of your interests and daily activities by experimenting with lifestyle changes designed to minimize your symptoms.

Where to Get More Information

For more information, try these online resources.

• Pulmonary Hypertension Association
• The National Heart, Lung, and Blood Institute of the National Institutes of Health
• Pulmonary Hypertension: The Complete Resource
• The American Lung Association