How Is Pulmonary Hypertension Treated?

Pulmonary hypertension (PH) has no cure. However, 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 its severity. (For more information, go to "Types of Pulmonary Hypertension.")

Group 1 Pulmonary Arterial Hypertension

Group 1 pulmonary arterial hypertension (PAH) includes PH that's inherited, that has no known cause, or that's caused by certain drugs or conditions. Treatments for group 1 PAH include medicines and medical procedures.

Medicines

Your doctor may prescribe 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.

Your doctor may prescribe medicines that are taken by mouth, inhaled, or injected.

Examples of medicines for group 1 PAH include:

• Phosphodiesterase-5 inhibitors, such as sildenafil
• Prostanoids, such as epoprostenol
• Endothelin receptor antagonists, such as bosentan and ambrisentan
• Calcium channel blockers, such as diltiazem

Your doctor may prescribe one or more of these medicines. To find out which of these medicines works best, you'll likely have an acute vasoreactivity test. This test shows how the pressure in your pulmonary arteries reacts to certain medicines. The test is done during right heart catheterization.

Medical and Surgical Procedures

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

Atrial septostomy (sep-TOS-toe-me). 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 (the upper chambers of your heart). This wall is called the septum.

A tiny balloon on the tip of the tube is inflated. This creates 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

Conditions that affect the left side of the heart, such as mitral valve disease, can cause group 2 PH. Treating the underlying condition will help treat PH. Treatments may include lifestyle changes, medicines, and surgery.

Group 3 Pulmonary Hypertension

Lung diseases, such as COPD (chronic obstructive pulmonary disease) and interstitial lung disease, can cause group 3 PH. Certain sleep disorders, such as sleep apnea, also can cause group 3 PH.

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. Oxygen therapy can be done at home or in a hospital.

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

Group 4 Pulmonary Hypertension

Blood clots in the lungs or blood clotting disorders can cause group 4 PH. If you have this type of PH, your doctor will likely prescribe blood-thinning medicines. These medicines prevent clots from forming or getting larger.

Sometimes doctors use surgery to remove scarring in the pulmonary arteries due to old blood clots.

Group 5 Pulmonary Hypertension

Various diseases and conditions, such as thyroid disease and sarcoidosis, can cause group 5 PH. An object, such as a tumor, pressing on the pulmonary arteries also can cause group 5 PH.

Group 5 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:

• Diuretics, also called water pills. These medicines help reduce fluid buildup in your body, including swelling in your ankles and feet.
• Blood-thinning medicines. These medicines help prevent blood clots from forming or getting larger.
• Digoxin. This medicine helps the heart beat stronger and pump more blood. Digoxin sometimes is used to control the heart rate if abnormal heart rhythms, such as atrial fibrillation or atrial flutter, occur.
• Oxygen therapy. This treatment raises the level of oxygen in your blood.
• Physical activity. Regular activity may help improve your ability to be active. Talk with your doctor about a physical activity plan that's safe for you.

Research is ongoing for better PH treatments. These treatments offer hope for the future.

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