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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. These arteries can narrow or become blocked or damaged. When this happens, they cannot carry as much blood to your lungs.  This causes pressure to build up in the narrowed arteries.  This puts strain on your heart because it has to work harder to push the blood through. Over time, this weakens your heart, and you can develop heart failure.

SYMPTOMS

What are the symptoms of pulmonary hypertension?

Pulmonary hypertension may not cause any symptoms at first. Often, shortness of breath or light-headedness during activity is the first symptom. As the disease gets worse, symptoms can include the following:

• Increased shortness of breath, with or without activity
• Fatigue (tiredness)
• Chest pain or pressure
• Rapid heartbeat
• Pain in the upper right side of the abdomen
• Decreased appetite
• Dizziness or fainting
• Swelling of the ankles, legs and abdomen
• Bluish tint or shading to your skin or lips

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
• Blood clots in the pulmonary arteries
• HIV infection
• Liver disease/cirrhosis
• Connective tissue diseases such as lupus or scleroderma
• Pulmonary fibrosis (a condition that causes scarring in the lungs)
• Certain medicines or street drugs

Some people have a higher risk of developing pulmonary hypertension. These include people who:

• Have a family history of the condition
• Have certain conditions, such as heart disease, lung disease, liver disease, HIV infection, or blood clots in the pulmonary arteries
• Use street drugs (such as methamphetamines or cocaine) or certain diet medicines
• Live at high altitudes

DIAGNOSIS & TESTS

How is pulmonary hypertension diagnosed?

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
• 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.  These could include:

• Blood tests
• A chest CT scan
• A chest MRI

If your healthcare provider determines that you have pulmonary hypertension, they will want to see how severe it is. For this, they may order an exercise test. These tests measure your activity level and how well your lungs and heart work while you are exercising. These tests can also be done during treatment to see how well the treatment is working.

PREVENTION

Not all pulmonary hypertension can be prevented. But you can try to prevent other conditions that can cause the disease. These include high blood pressure, heart disease, liver disease, and chronic lung disease from tobacco use.

TREATMENT

How is pulmonary hypertension treated?

There is no cure for pulmonary hypertension. But treatment can improve symptoms and slow the progression of the disease. The kind of treatment you receive could depend on what is causing the condition.  If the cause of your pulmonary hypertension is known, treating the cause may improve your condition. For example, if your condition is caused by a lung disease, you may need oxygen therapy to raise the level of oxygen in your blood. Or if it is caused by blood clots in your lungs, you will likely take blood-thinning medicines to prevent clots from getting larger.

There are several treatments that are often used to treat pulmonary hypertension no matter what the cause. These include:

• Diuretics (water pills). These reduce fluid build-up in your body.
• Blood-thinners. These help prevent blood clots from forming or getting bigger.
• Oxygen therapy. This raises the level of oxygen in your blood.
• This medicine helps the heartbeat stronger and pump more blood.
• Other medicines. Medicine that relaxes the blood vessels allows more blood to flow

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.  Regular activity can help improve your ability to be active.

In some severe cases, people who have pulmonary hypertension need surgical treatment. This could include a lung transplant or a heart and lung transplant.

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

Living with pulmonary hypertension

Because there is no cure for pulmonary hypertension, you will need to learn to manage your condition. The best way to do this is by following the treatment plan developed between you and your doctor. Call your doctor if your symptoms change or get worse. Know what symptoms could require emergency treatment, such as chest pain.

Making lifestyle changes can also help your condition. This could include:

• Quitting smoking. Smoking makes symptoms of pulmonary hypertension worse.
• Following a healthy diet. Eat a variety of fruits, vegetables, whole grains and lean protein. Talk to your healthcare provider to see if you need to limit the amount of salt in your diet. Also ask if you should limit the amount of vitamin K in your diet. Vitamin K is found in green leafy vegetables and some oils. It can affect how well blood-thinning medicines work.
• Being physically active. Try to be as active as you can. Incorporate regular activity, such as walking, into your daily routine. Ask your healthcare provider if there are any activities you should avoid. These could include lifting heavy objects, sitting in a hot tub, or traveling to high-altitude areas.
• Getting support for emotional issues. Living with pulmonary hypertension can cause you to have anxiety, stress, or depression. This could include talking to a counselor, medicine for depression, or a patient support group.

Which changes you should make may depend on the cause of your condition. Talk to your doctor about what types of changes are best for you.