How Is Pulmonary Arterial Hypertension Treated?

Reviewed by: HU Medical Review Board

Pulmonary arterial hypertension (PAH) is high blood pressure in the arteries of the lungs. The condition narrows, blocks, or destroys arteries in the lungs. This makes the heart work harder than normal to push blood through the lungs. The extra work puts strain on the heart. Eventually, the heart muscle becomes weak and fails.1

PAH is a rare subtype of pulmonary hypertension. Experts estimate that about 10 of every 1 million adults in the United States have PAH. There is no cure for PAH. But there are many treatments that reduce symptoms and keep the disease from getting worse.1,2

Types of PAH

There are different subtypes of PAH. Each subtype may be treated differently. They include:1,2

  • Idiopathic PAH: Basically PAH with an unknown cause.
  • Heritable PAH: This type of PAH runs in families. It accounts for up to 1 in 10 cases among all people with PAH.
  • Drug- and toxin-induced PAH: PAH caused by certain prescription and illegal drugs. Examples include methamphetamine, dasatinib, or fenfluramine.
  • PAH linked to other health conditions: PAH caused by another disease, such as:
    • Connective tissue disease (scleroderma, lupus)
    • HIV
    • Cirrhosis of the liver
    • Congenital heart disease

There are other rare subtypes of PAH.2

PAH treatment options

Your doctor will recommend treatments based on what is causing your PAH and how severe your symptoms are. Some options include:1-4

  • Medicines
  • Oxygen therapy
  • Lifestyle changes
  • Surgery
  • Treatment for other health conditions


Many drugs may be prescribed for PAH, alone or in combination. Not all of these medicines are right for every person with PAH. Some cannot be taken during pregnancy, for instance. Some may be used for other health conditions such as erectile dysfunction and irregular heartbeat. Drugs used to treat PAH include:1,2,4

  • Blood vessel dilators (vasodilators) relax and open narrow blood vessels. Examples include:
    • Epoprostenol (Flolan®, Veletri®)
    • Treprostinil (Tyvaso®, Remodulin®, Orenitram®)
  • Guanylate cyclase stimulators lower blood pressure in the lungs by relaxing lung arteries. Riociguat (Adempas®) is one example.
  • Endothelin receptor antagonists widen blood vessels. Examples include:
    • Ambrisentan (Letairis®)
    • Bosentan (Tracleer®)
    • Macitentan (Opsumit®)
  • Phosphodiesterase 5 inhibitors increase blood flow in the lungs. Examples include:
    • Sildenafil (Revatio®, Viagra®)
    • Tadalafil (Adcirca®, Cialis®, Alyq™)
  • Calcium channel blockers relax the muscles in the walls of your blood vessels. Examples include:
    • Amlodipine (Norvasc®)
    • Diltiazem (Cardizem®, Tiazac®)
    • Nifedipine (Procardia®)
  • Blood thinners (anticoagulants) help to keep blood clots from forming. One example is warfarin (Jantoven®).
  • Digoxin (Lanoxin™) helps the heart pump more blood.
  • Water pills (diuretics) help the kidneys flush fluid from your body when there is too much. This eases how hard the heart has to work.

Oxygen therapy

Some people may benefit from pure oxygen. This is especially true for people with PAH who:1,2,4

Lifestyle changes

Some people need to make changes to their lifestyle to help control the symptoms of PAH. Common suggestions include:2-4

  • Eating less salt and a heart-healthy diet
  • Exercising as much as possible – but avoid lifting weights
  • Exercising with a specialist to build lung function
  • Getting plenty of rest
  • Stopping smoking
  • Avoiding high altitudes
  • Avoiding activities that lower blood pressure, for example:
    • Not spending time in hot tubs or saunas
    • Not taking long, hot showers and baths
  • Getting recommended vaccines, especially ones that help prevent lung infections
  • Talking to your doctor before becoming pregnant or if you are taking birth control pills


When medicines and lifestyle changes are not enough to control PAH symptoms, your doctor may recommend surgery. Types of surgery that may help include:2-4

  • Atrial septostomy. A type of open heart surgery that creates an opening between the upper chambers on the left and right sides of the heart.
  • Lung or heart-lung transplant. This is most often an option for people with idiopathic PAH who are younger.

Treatment for other health conditions

With PAH, it is important to closely manage all of your health conditions. That is especially true if your other health conditions are causing or contributing to PAH. This means your doctor may prescribe:1,3

  • Blood transfusions or hydroxyurea for sickle cell disease
  • Heart valve repair
  • Iron for anemia
  • Treatment of connective tissue or liver diseases
  • Heart surgery to correct heart defects

PAH is a serious, complex medical condition that requires specialty care. That is why anyone diagnosed with or suspected to have PAH should be seen in a clinic with doctors who have experience treating this rare condition. Treatment can slow the progression of PAH and improve your quality of life.4

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