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Treating and Managing Pulmonary Arterial Hypertension

How Pulmonary Arterial Hypertension Is Treated

Although there is no cure for PAH, there are medications and procedures that can slow the progression of the disease and improve your quality of life. Treatment options vary from person to person, so you will want to work closely with your doctor to determine the best plan for you. Newly diagnosed patients with PAH should be referred to an accredited pulmonary hypertension care center for evaluation and right heart catheterization.

PAH-specific medications come in multiple forms: oral, inhaled and intravenous (IV)/subcutaneous. Intravenous treatments open up the blood vessels to help ease chest pain and shortness of breath. Subcutaneous treatments use a portable infusion pump to open up the blood vessels and ease PH symptoms. Inhaled treatments, such as prostacyclins, are primarily used to relieve shortness of breath.

There are a wide range of oral treatment options. Endothelin Receptor Antagonists (ERAs) prevent blood vessels from narrowing and are commonly used to help patients participate in physical activities. Phosphodiesterase Inhibitors (PDE 5 Inhibitors) allow the lungs to relax and the blood vessels to dilate. Prostacyclin Analogue, Selective IP Receptor Agonist, and Soluble Guanylate Cyclase (sGC) Stimulators allow the blood vessels in the lungs relax.

In addition, a number of supportive therapies are recommended in conjunction with medication. Diuretics can help treat fluid retention and swelling. Anticoagulants may be prescribed to prevent blood clots from forming. Some patients may require a portable oxygen tank. In the most severe cases, a heart-lung, single lung or double lung transplant may be recommended. Pulmonary rehabilitation as well as daily exercise are also highly recommended to help patients improve breathing and quality of life.

Managing Pulmonary Arterial Hypertension

Being diagnosed with a chronic illness like PAH is life-changing. Patients will need to have a close relationship with a PAH expert as well as an excellent support system of family and friends.

Many pulmonary hypertension care centers require visits every few months and regular testing such as echocardiograms and 6-minute walk testing. Some centers will do right heart catheterizations every year to see how well treatment is working on pulmonary pressures and heart function. Additionally, it is essential to take your medications exactly as directed, being careful not to run out or change your schedule unless directed by your doctor. It is also important to get yearly flu and pneumonia vaccinations.

PAH patients should adopt or continue these lifestyle changes to maintain their health.

  • Quitting smoking and avoiding secondhand smoke is one of the most important decisions you can make. The American Lung Association offers great resources to help you quit. 
  • Though PAH may make you feel fatigued, it is important to stay active and exercise as much as possible. You may need to avoid activity such as weight lifting that can put too much strain on your lungs and heart. Ask your doctor for help creating a safe exercise plan.
  • Avoid sitting in a hot tub or sauna, as well as traveling to high altitudes, as that can also put strain on your lungs and heart.
  • Eating healthy will be an important part of managing your disease. Many doctors suggest avoiding saturated fat, trans fat and cholesterol and limiting your salt intake.
  • Pregnancy can put strain on your body and for a patient with PAH be possibly life-threatening, so this is a subject you should discuss with your doctor prior to becoming pregnant.    
  • With all this change, patients with PAH can easily feel overwhelmed. Many individuals may experience anxiety and/or depression after diagnosis. There are a number of support groups and other mental healthcare options available. If you are having problems coping with your situation, talk to your doctor about getting some help.
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Finding Support

The Lung Association recommends patients and caregivers join our Living with PAH Support Community to connect with others facing this disease. Join a Better Breathers Club to learn better ways to cope with your PAH diagnosis. You can also call the Lung Association's Lung Helpline at 1-800-LUNGUSA to talk to a trained respiratory professional who can help answer your questions and connect you with support.

Page last updated: March 17, 2020

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