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Primary Pulmonary Hypertension

  • Pulmonary arteries and veins

Definition

Pulmonary hypertension refers to high blood pressure in the arteries that lead from the right side of the heart to the lungs. If no other cause can be found, the condition is called primary pulmonary hypertension.

What is going on in the body?

The pulmonary blood pressure is the pressure in the arteries in the lungs. This is not the same as the blood pressure that is measured in a doctor's office. Pulmonary pressure can only be measured by special tests, such as an echocardiogram, or in the cardia catheterization lab. The blood pressure in the pulmonary arteries can be high for many reasons. In some cases, no cause for the increased pressure is found. This is known as primary pulmonary hypertension, which is rare.

Risks

What are the causes and risks of the disease?

The cause of this unusual condition is unknown. Some cases are thought to be inherited. This condition is most commonly seen in women between the ages of 20 and 40 years. Usually, primary pulmonary hypertension results in a weakening of the heart and eventual death.

Prevention

What can be done to prevent the disease?

There are no known ways to prevent this condition.

Diagnosed

How is the disease diagnosed?

Pulmonary hypertension is usually caused by other conditions. Extensive testing of the heart and lungs is needed to rule out these causes. The tests include blood tests, breathing tests, and special x-ray tests. Occasionally, it is necessary to remove a piece of lung tissue with a special needle and examine it under a microscope (a lung biopsy). If no other cause can be found, the diagnosis of primary pulmonary hypertension is made.

Long Term Effects

What are the long-term effects of the disease?

This disease causes severe weakening of the heart and leads to death. A person usually survives only 2 to 3 years after the diagnosis has been made.

Other Risks

What are the risks to others?

This condition is not contagious and poses no risk to others.

Treatments

What are the treatments for the disease?

The only cure for primary pulmonary hypertension is a lung transplant to replace both lungs. Sometimes a heart transplant is also needed. Medications can be used to improve symptoms for a short time, but the person usually dies unless he or she receives a lung transplant.

Side Effects

What are the side effects of the treatments?

All medications have possible side effects. These may include allergic reactions and low blood pressure. Lung and heart transplants are major operations that can result in bleeding, infection, and death.

After Treatment

What happens after treatment for the disease?

A person will generally need lifelong treatment. If a lung transplant is not done, the types of medications and the doses will need to increase as the condition gets worse.
After a transplant, the person must take medication to prevent his or her immune system from rejecting the lung. These drugs have significant side effects. Intense monitoring is also needed after a transplant.

Monitor

How is the disease monitored?

A person with primary pulmonary hypertension needs lifelong monitoring with blood tests, breathing tests, special blood pressure measurements, and various x-ray tests. These patients are best followed at a major medical center that has specialists in this disorder.

Sources

Harrison's Principles of Internal Medicine, 1998, Fauci et al.

Cecil Textbook of Medicine, 1996, Bennett et al.

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