How Is Pulmonary Hypertension Treated?

Pulmonary hypertension (PH) has no cure, but treatment may help relieve symptoms and slow the progress of the disease.

PH is treated with medicines, procedures, and other therapies. Treatment will depend on what type of PH you have and how severe it is.

 

Group 1 Pulmonary Arterial Hypertension

This type of PH includes PH that's inherited, that has no known cause, or that's caused by certain conditions. Treatment for group 1 pulmonary arterial hypertension (PAH) includes medicines or medical procedures.

 

Medicines

 

If you have group 1 PAH, you may need medicines to relax the blood vessels in your lungs and reduce excess cell growth in the blood vessels. As the blood vessels relax, more blood can flow through them.

 

Examples of these medicines include phosphodiesterase-5 inhibitors, prostanoids, endothelin receptor antagonists, and calcium channel blockers.

 

To find out which of these medicines will work best, you'll likely have an acute vasoreactivity test. This test shows how the pressure in your pulmonary arteries reacts to certain medicines. This test is done during right heart catheterization.

 

Medical and Surgical Procedures

 

If you have group 1 PAH, you may need one or more of the following procedures.

 

Atrial septostomy. For this procedure, a thin, flexible tube called a catheter is put into a blood vessel in your leg and threaded to your heart. The tube is then put through the wall that separates your right and left atria. This wall is called the septum.

A tiny balloon on the tip of the tube is inflated to create an opening between the atria. This procedure relieves the pressure in the right atria and increases blood flow. Atrial septostomy is rarely done in the United States.

 

Lung transplant. A lung transplant is surgery to replace a person's diseased lung with a healthy lung from a deceased donor. This procedure may be used for people who have severe lung disease that's causing PAH.

 

Heart–lung transplant. A heart–lung transplant is surgery in which both the heart and lung are replaced with healthy organs from a deceased donor.

 

Group 2 Pulmonary Hypertension

 

Group 2 PH is caused by conditions that affect the left side of the heart, such as mitral valve disease. Treating these conditions also will help treat PH. Treatments may include lifestyle changes, medicines, and surgery.

 

Group 3 Pulmonary Hypertension

 

Group 3 PH is linked to lung conditions, such as COPD (chronic obstructive pulmonary disease) and interstitial lung disease. Group 3 PH also may be linked to certain sleep disorders, such as sleep apnea.

If you have this type of PH, you may need oxygen therapy. This treatment raises the level of oxygen in your blood. You'll likely get the oxygen through soft, plastic prongs that fit into your nose. You can get oxygen therapy at home or in a hospital.

Your doctor also may recommend other treatments if you have an underlying lung disease.

 

Group 4 Pulmonary Hypertension

 

This type of PH is due to blood clots in the lungs, blood clotting disorders, or sickle cell anemia. If you have this type of PH, your doctor will likely prescribe anticoagulants, or "blood thinners." These medicines prevent clots from forming or from getting larger.

In some cases, surgery is used to remove scarring in the pulmonary arteries due to old blood clots.

 

Group 5 Pulmonary Hypertension

 

Various diseases and conditions cause group 5 PH. Examples include sarcoidosis, Langerhans cell histiocytosis, and lymphangioleiomyomatosis (LAM). This type of PH also may be due to an object, such as a tumor, pressing on the pulmonary blood vessels.

This type of PH is treated by treating its cause.

 

All Types of Pulmonary Hypertension

 

Several treatments may be used for all types of PH. These treatments include:

 

Research on new treatments for PH is ongoing. These treatments offer hope for the future. Talk to your doctor about whether you should consider participating in research studies.

 

"Strength does not come from physical capacity. It comes from an indomitable will."

 

Living With Pulmonary Hypertension

Pulmonary hypertension (PH) has no cure. However, you can work with your doctor to manage your symptoms and slow the progress of the disease.

 

Ongoing Care

 

Follow your treatment plan and call your doctor if your PH symptoms worsen or change. The earlier symptoms are addressed, the easier it is to treat them. Some symptoms, such as chest pain, may require emergency treatment. Talk to your doctor about when you should call him or her and when to seek emergency care.

 

Also, talk to your doctor before taking any over-the-counter medicines. Certain medicines can make your PH worse or interfere with the medicines you're taking for PH. Ask your doctor whether you should get a pneumonia vaccine and a yearly flu shot.

 

Know the names of your medicines and how they work. Keep a list of your medicines with you. Don't stop or change your medicines unless you talk with your doctor.

 

Pay careful attention to your weight. You may want to keep a daily record of your weight. You should weigh yourself at the same time each day. If you notice a rapid weight gain (2 or more pounds in 1 day or 5 or more pounds in 1 week), call your doctor. This may be a sign that your condition is worsening.

 

Pregnancy is risky for women who have PH. Consider using birth control if there's a chance you may become pregnant. Talk to your doctor about which birth control methods are right for you.

Lifestyle Changes

Making lifestyle changes can help you manage your symptoms. These changes will depend on the type of PH you have. Talk to your doctor about which steps can help you.

 

Quit Smoking

 

If you smoke, quit. Smoking makes PH symptoms worse. Ask your doctor about programs and products that can help you quit. Also, avoid exposure to secondhand smoke.

 

Follow a Healthy Diet

 

Follow a healthy diet and maintain a healthy weight. A healthy diet includes a variety of fruits, vegetables, and whole grains. It also includes lean meats, poultry, fish, and fat-free or low-fat milk or milk products. A healthy diet also is low in saturated fat, trans fat, cholesterol, sodium (salt), and added sugar.

 

Talk to your doctor about whether you need to limit the amount of salt and fluids in your diet. Ask him or her whether you also need to regulate foods that contain vitamin K. These foods can affect how well blood-thinning medicines work. Vitamin K is found in green leafy vegetables and some oils, such as canola and soybean oil.

 

Do Physical Activity

 

Do regular physical activity, such as walking. This will keep your muscles strong and help you stay active. Talk to your doctor about how much activity is safe for you. Your doctor may tell you to limit or avoid certain activities, such as:

 

Avoid activities that cause breathing problems, dizziness, or chest pain. If you have any of these symptoms, seek care right away.