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OVERVIEW

What is pulmonary hypertension?

“Pulmonary” means “in the lungs,” and “hypertension” means “high blood pressure.” Pulmonary hypertension is an increase in blood pressure in the blood vessels that carry blood to the lungs. It is a serious health problem.

Pulmonary arteries are the blood vessels that carry blood from your heart to your lungs. If these arteries narrow or become blocked or damaged, they cannot carry as much blood. This puts strain on your heart because it has to work harder to push blood through your lungs. Pressure builds up in the narrowed arteries. This results in high blood pressure in the right side of your heart and in the blood vessels that carry blood to the lungs.

SYMPTOMS

What are the symptoms of pulmonary hypertension?

Pulmonary hypertension may not cause any symptoms at first. As the disease gets worse, symptoms can include the following:

• Shortness of breath, with or without activity
• Cough
• Fatigue
• Dizziness
• Chest pain or pressure
• Rapid heartbeat
• Swelling of the ankles, legs and abdomen

Symptoms of pulmonary hypertension usually limit a person’s ability to exercise and do other activities.

CAUSES & RISK FACTORS

What causes pulmonary hypertension?

Many things can cause pulmonary hypertension. However, sometimes the cause isn’t known. When the cause of pulmonary hypertension isn’t known, it is called idiopathic pulmonary hypertension (IPH).

When pulmonary hypertension develops because of another medical condition, it is called secondary pulmonary hypertension. Breathing problems such as emphysema and chronic bronchitis, as well as sleep apnea, are common causes of secondary pulmonary hypertension. Other causes include the following:

• Congestive heart failure
• Birth defects in the heart
• Chronic pulmonary thromboembolism (blood clots in the pulmonary arteries)
• Acquired immunodeficiency syndrome (AIDS)
• Cirrhosis(a chronic liver disease)
• Lupus
• Pulmonary fibrosis (a condition that causes scarring in the lungs)
• Certain medicines

DIAGNOSIS & TESTS

How does my healthcare provider know that I have pulmonary hypertension?

Signs of pulmonary hypertension can be similar to the signs of many other health problems. This makes it hard to diagnose. Your healthcare provider will probably run some tests to estimate the blood pressure in your pulmonary arteries and find out how well your heart and lungs are working. These tests may include a chest X-ray, a breathing test called a pulmonary function test and an echocardiogram (sometimes called an “echo”). Your healthcare provider may also need to do other tests to find out whether another medical condition is causing your pulmonary hypertension.

TREATMENT

How is pulmonary hypertension treated?

If the cause of your pulmonary hypertension is known, treating the cause may improve your condition. Lifestyle changes can also help you feel better. If you smoke, stop. Maintain a healthy weight, eat a nutritious diet and reduce the amount of stress in your life. If you are a loud snorer or have other signs of sleep apnea, ask your healthcare provider about a sleep study to diagnose this condition. Ask your healthcare provider to recommend ways that you can stay as active as possible.

Breathing oxygen from a tank can help relieve shortness of breath. Medicines that can be used to treat pulmonary hypertension include the following:

• Endothelin receptor antagonists
• Phosphodiesterase-5 inhibitors
• Prostacyclins
• Anticoagulants (blood-thinning medicine)
• Calcium channel blockers
• Diuretics (water pills)
• Digoxin

Your healthcare provider will decide what type of medicine is right for you.

In some cases, people who have pulmonary hypertension need surgical treatment. Surgical treatment options include a lung transplant and a type of heart surgery called atrial septostomy (say: “ay-tree-allsep-toss-teh-mee”).

QUESTIONS TO ASK YOUR HEALTHCARE PROVIDER

• How do you know what’s causing my pulmonary hypertension?
• Is the underlying condition treatable?
• What kind of tests will I need? Are they covered by insurance?
• Is it safe for me to exercise? What kinds of exercise can I do?
• I have other health problems. Will treatment of pulmonary hypertension affect the treatment of my other health problems?
• Will I need surgery? What does surgery entail?

Sources

Diagnosis and Treatment of Pulmonary Hypertension by TD Nauser, MD; SW Stites, MD (05/01/01, http://www.aafp.org/afp/20010501/1789.html)