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Pulmonary arterial hypertension (PAH) is one of five types of pulmonary hypertension. It happens when the blood vessels in your lungs narrow and scar so much that it’s hard for blood to move through them. This can trigger high blood pressure in the lungs.

PAH can be hard to diagnose. That’s because the symptoms like shortness of breath are common to several other medical conditions. There’s no cure. But there are FDA-approved treatments. Here’s what you need to know.

How Is Pulmonary Arterial Hypertension Treated?

What caused your PAH, the symptoms, and how advanced your condition is all impact what treatment might work best for you. It may take time to figure out, but your doctor is there to help.  

Options include:

  • Medications
  • Surgery
  • Oxygen therapy
  • Calcium channel blockers
  • Blood thinners
  • Diet changes
  • Exercise

What Medications Can I Take for Pulmonary Arterial Hypertension?

Prescription drugs called vasodilators specifically treat PAH. These may be taken by mouth or given through an IV or subcutaneously, meaning under the skin. Some you inhale, or breathe in, from a nebulizer.

In some cases, you may need more than one type of drug to get your condition under control. You might also need other therapies like extra oxygen along with diet and lifestyle changes to help you feel better.

Intravenous (IV) treatments

Epoprostenol is a vasodilator that relaxes your blood vessels so it’s easier for blood to move through them. There are two brands: Flolan and Veletri.

Flolan. In 1995, this was the first drug the FDA approved to treat PAH. It’s given as a continuous injection through a vein for short-term use. A doctor can also surgically place a special catheter for long-term use. One study found that it helped make exercise easier and boosted survival rates.

Flolan has to be mixed every day. You must also:

  • Refrigerate the medicine when you’re not using it
  • Use ice packs to keep it cold during infusion
  • Have your blood count checked regularly

Your catheter also needs to be checked often for signs of possible infection.

Side effects may include:

  • Headache
  • Diarrhea
  • Nausea
  • Jaw pain
  • Flushing of the skin
  • Dizziness
  • Swelling
  • Itching
  • Muscle or joint pain
  • Low systemic blood pressure

Selexipag IV (Uptravi). It can help slow the progression of PAH and lower your risk of being hospitalized. It's used only in patients who temporarily can't take oral medication. 

Treprostinil (Remodulin). This is another IV formula of the same class of drugs as Flolan and Veletri.  It’s also stable at room temperature and doesn’t have to be mixed daily. It can also be given under the skin. 

Veletri. It’s a newer version of epoprostenol approved by the FDA in 2010. It doesn’t have to be mixed daily, and you can keep it at room temperature. Your doctor may suggest Veletri if you have severe PAH and other treatments haven’t worked.

Side effects for Veletri and Remodulin are similar to those of Flolan. Catheters used for IVs should be checked regularly to avoid infections.

Nebulizer treatments

Iloprost (Ventavis).  You breathe it in through a nebulizer about six to nine times every day. If you have asthma or other conditions that can make it hard to breathe, this may worsen those symptoms.

Treprostinil (Tyvaso). Your doctor may recommend this drug if you have moderate to severe PAH symptoms. You’ll likely take it about four times a day. Research suggests it can help you walk longer distances.

Side effects of iloprost and treprostinil are similar to those of  IV-based PAH drugs. But you may have less flushing or diarrhea with iloprost. Treprostinil may cause a sore throat and cough when you first start to use it.

Oral treatments

Ambrisentan (Letairis). This is a  once-a-day medication, but unlike bosentan, it doesn’t affect your liver.

Bosentan (Tracleer). This medication is taken two times a day. The drug could affect your liver function and hemoglobin levels. It’s important to get your blood count and liver function tested regularly when you’re taking bosentan.

Neither bosentan nor ambrisentan is recommended for people who are pregnant. Experts recommend two separate birth control options. Monthly pregnancy tests are required to continue using these drugs.

Sildenafil (Revatio). Small studies suggest this drug can improve blood flow in your lungs. The recommended dose is a 20-milligram pill taken three times a day, but it can be increased if you have a more severe form of PAH.

Side effects of sildenafil include:

  • Headache
  • Back pain
  • Flushing
  • Possible drug interactions

Tadalafil (Adcirca). This is a once-a-day pill prescribed if you have moderate to severe PAH.

Treprostinil (Orenitram) is the oral formulation of Remodulin and can substitute for the IV medications after you have stabilized.

Other Ways to Treat Pulmonary Arterial Hypertension

These include:

Calcium channel blockers. This class of drugs, in select patients, can help lower the blood pressure in your lungs.

Blood thinners. Your risk of forming blood clots is higher if you have PAH. Blood thinners, like warfarin (Coumadin), are given to most people with PAH to cut down on clots and improve overall survival. But warfarin interacts with several foods and drugs. Your doctor can tell you what to avoid.

Diuretic. Sometimes called water pills, diuretics can help your body get rid of extra salt and water. This happens to most people with PAH. Your doctor will usually recommend one pill a day. But if you have a severe form of PAH, they may up the dosage.

Digoxin. This is an older medication sometimes prescribed to people with severe PAH. It’s designed to help your heart pump blood better.

Oxygen therapy

When you have moderate to severe PAH, it can make it hard to breathe properly and affect your day-to-day life. The low levels of oxygen can make the blood vessels constrict further, which can make your PAH worse.

Getting a little extra oxygen can help you not only breathe better but also be able to get through daily tasks and routines. It can also boost your mood and sleep. While some may need extra oxygen every now and then, others may need it continuously.

Oxygen therapy comes in three types:

  • Compressed gas oxygen. This is in steel or aluminum cylinders that you can use at home or when you travel.
  • Liquid oxygen
  • Oxygen concentrators. This device removes nitrogen from the air to provide a concentrated form of oxygen to breathe.

Before you try oxygen therapy, ask your doctor how much you need and how often you may need it. They can also guide you toward the type that works best for your lifestyle.

Surgery

If you have severe PAH and medications aren’t working, your doctor may recommend a lung transplant. Lung transplant surgery is challenging and can come with serious complications. These include:

  • Infection
  • Kidney failure
  • Organ rejection

 Your health care team can help you decide if it’s right for you.

Things You Can Do to Manage Pulmonary Arterial Hypertension

There are some lifestyle changes you can try to help ease your symptoms.

You can:

  • Get plenty of rest to ease fatigue.
  • Don’t smoke, or try to quit if you do.
  • Avoid travel to high altitudes.
  • Avoid activities that can lower your blood pressure, like long, hot baths or saunas.
  • Eat a balanced diet rich in whole grains, vegetables, and fruits, and avoid too much salt.
  • Stick to your treatment plan. Don’t make any changes without running them by your doctor.
  • Get recommended vaccines to avoid seasonal lung infections.

Try to exercise and stay as active as you can. But don’t lift heavy weight or do intense workouts. Talk to your doctor first if you’re not sure what’s safe.

Show Sources

Photo Credit: Charles Wollertz / Getty Images

SOURCES:

Mayo Clinic: “Pulmonary hypertension,” “Epoprostenol (Intravenous Route).”

Chest Foundation: “Pulmonary Arterial Hypertension (PAH).”

Pulmonary Hypertension Association: “About Pulmonary Hypertension,” “Treatments,” “Epoprostenol (Flolan).”

Stanford Medicine: “FDA-approved Treatments for Pulmonary Hypertension.”

Cleveland Clinic: “Connective Tissue Diseases.”