What Is Pulmonary Hypertension?

When the pressure in the blood vessels leading from your heart to your lungs is too high, you may feel tired. This is called pulmonary hypertension.

What is pulmonary hypertension?

To get oxygen into your blood, your heart pumps it from the right ventricle to your lungs. That’s not a long way, so the pressure in this side of your heart and in the artery involved tends to be low. It should be much lower than either your systolic or diastolic blood pressure.

If you have pulmonary hypertension (PH), which affects about 1 percent of all people, the walls of the blood vessels leading to your lungs become more muscular, causing the pressure in the artery to rise. Eventually, the artery may narrow, which makes it hard for blood to flow through. The result is blood with less oxygen.

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What are the signs and symptoms of pulmonary hypertension?

In the beginning, you might feel tired or have difficulty breathing. Over time, patients feel dizzy and may have chest pain or a racing heart.

What are the risk factors for pulmonary hypertension?

Although this problem becomes more common at age 75 or older, it can happen to children. Women and black people are especially vulnerable.

What causes pulmonary hypertension?

It’s common for PH to be linked to another illness. You might have a congenital heart disease, connective tissue disease, high blood pressure, liver disease (cirrhosis), blood clots to the lungs, or emphysema. Genetics also play a role. About half of patients with heart failure have PH.

Five subgroups or types of PH grouped by the cause:

• Pulmonary arterial hypertension (PAH)
• PH from left-sided heart disease
• PH from chronic lung disease
• Chronic thromboembolic PH (CTEPH)
• PH with an unclear mechanism or multifactorial mechanisms

A range of underlying conditions can lead to these disorders.

How is pulmonary hypertension diagnosed?

Your doctor might ask for electrocardiography, chest radiography, pulmonary function tests, or transthoracic echocardiography. A ventilation-perfusion scan will rule in or exclude CTEPH. Right-sided heart catheterization is essential for accurate diagnosis and classification.

How is pulmonary hypertension treated?

You can find relief with medication, which may be inhaled or injected into the skin. Receiving oxygen or medicine to cut swelling in your feet may make you more comfortable.

Previously regarded as untreatable, the treatment of PAH has dramatically advanced since the introduction of the drug epoprostenol in 1999, with three-year survival rates improving from 30 to 40 percent to over 85 percent. Other more recent drugs are now available.

In the past decade, management and treatment of CTEPH have also improved. Pulmonary endarterectomy, an operation to remove old blood clots and scar tissue from the pulmonary arteries in the lungs, used to be the only option for CTEPH. Newer treatments now include balloon pulmonary angioplasty, which uses balloons to open blocked or narrowed blood vessels, and a medication called a soluble guanylate cyclase stimulator.

Management of PH resulting from left-sided heart disease primarily involves treatment of the underlying condition.

How to prevent pulmonary hypertension

Do your best to avoid or manage any linked illnesses, such as high blood pressure, coronary heart disease, chronic liver disease, and chronic lung disease from tobacco use.

Living with pulmonary hypertension

Many people can’t keep up with all the activities they once could pursue. For some people, a task that once took an hour may take several days or more. Pace yourself, and make a point of finding ways to feel good about what you can do.

It helps to make a list of your main activities and rank them in importance. You’ll need to let go of things that are less important. Your goals need to be realistic.

Organize your home so you don’t need to bend over or look for items often. For example, you might put a dryer on a stand so you don’t need to bend over to change loads. Raise your toilet about six inches. Order groceries online and use pick-up and delivery services. Use wheelchairs and handicapped parking — but first apply for a handicapped parking pass.

After you’ve learned how to minimize exertion, remember that your life isn’t all about being sick. Keep hobbies or develop new ones that require less energy. It’s rarely “all or nothing.” You might not be able to manage a large garden, but you can tend to house plants or window boxes.

Things you can do from home: writing, arts and crafts, genealogical research, reading. Helping others through volunteer work usually will make you feel stronger.

For more information:

• National Heart, Lung, and Blood Institute
• American Heart Association
• Pulmonary Hypertension Association

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