Treating and Managing PAH

Although there is no cure for pulmonary arterial hypertension, there are medications and treatment options that can slow the progression of the disease and improve your quality of life.

Studies indicate that early diagnosis is associated with improved survival rates and will help with getting started on the right treatment path. Treatment options vary from person to person, so you will want to work closely with your healthcare provider to determine the best plan for you.

Your Treatment Plan

If you are newly diagnosed with PAH, you should be referred to an accredited pulmonary hypertension care center for thorough evaluation. Because PAH is such a rare disease, it is extremely valuable to see a specialist at an accredited center to ensure you are getting the most up-to-date treatment options. 

Medications and Treatment Guide

PAH-specific medications come in multiple forms: oral, inhaled and subcutaneous (meaning delivered by an injection or IV). The medicines for PAH work in a few ways. Some allow blood to flow more easily through the arteries of your lungs. Others help your heart and lungs work better.

Treatment aims to restore balance among one or more of three substances that are produced by your lungs: nitric oxide, endothelin, and prostacyclin. Although a test does not currently exist to determine which of these substances is not balanced, PAH medications act on these three pathways to help slow how quickly your disease worsens.

Types of Medications to Manage PAH

Learn more about what each medication does, common side effects and additional information.
View Guide

Supportive Treatments 

Your healthcare provider may recommend supportive treatment to manage your PAH.  

One or more of the following medications may be recommended:

  • Blood thinners to prevent blood clots
  • Digoxin to help your heart beat regularly
  • Water pills, also called diuretics, to remove extra fluid from your body
  • Oxygen therapy to make sure you are getting enough oxygen

Learn more about PAH medications.

  • Pulmonary rehabilitation may improve exercise endurance, muscle strength and quality of life. 
  • Pulmonary rehabilitation is a program of education and exercise to increase awareness about your lungs and your disease 
  • The skills you learn in the program can help you better manage your disease

Learn more about pulmonary rehabilitation.

Surgery may be recommended in some situations.  

  • Atrial septostomy is a procedure that creates an opening between the upper left and right chambers of the heart. The opening reduces the pressure on the right side of the heart.
  • Lung or heart-lung transplant.

Managing Pulmonary Arterial Hypertension

Being diagnosed with a chronic illness like PAH is life-changing. You will need to have a close relationship with a PAH expert and would benefit from a strong 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. Your doctor may also have you complete cardiopulmonary exercise testing (CPET), a specialized type of exercise test that measures your exercise ability. Some centers will do right heart catheterizations every year to see how well treatment is working on managing 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 healthcare provider. It is also important to stay up to date on all recommended vaccinations.

Lifestyle Changes

PAH can affect every aspect of your daily life. No matter where you are at in your PAH journey, making lifestyle changes can help you better manage your health and your quality of life.

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 activities such as weightlifting that can put too much strain on your lungs and heart. Ask your healthcare provider for help creating a safe exercise plan and find out if pulmonary rehabilitation would benefit you.

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 healthcare providers suggest avoiding saturated fat, trans fat and cholesterol and limiting your salt intake. Talk to your healthcare provider or a registered dietitian to learn more about a healthy diet for you.

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 healthcare provider prior to becoming pregnant.

With all this change, you 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 healthcare provider about getting some help.

Finding Support

The Lung Association recommends patients and caregivers join our Living with PAH Support Community on Inspire.com to connect with others facing this disease. 

By joining a Better Breathers Club you can learn better ways to cope with your PAH diagnosis and have the support of others living with similar situations. 

You can also contact the Lung HelpLine to connect with a trained respiratory professional who can help answer your questions and connect you with support.

Page last updated: September 3, 2024

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