Early diagnosis of PAH 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 a pulmonary hypertension specialist and if possible be seen at an accredited pulmonary hypertension care center for thorough evaluation. Because PAH is such a rare disease, it is extremely valuable to see a specialist to ensure you are getting the most up-to-date treatment options.
Discover how self-advocacy and partnering with a specialist helped Jenna navigate PAH while staying focused on what matters most in her life.
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.
Free Medication Tracker App
Keeping track of medications can be challenging. Know when to take your medications, track side effects, get alerts when you need refills and more by using this FREE Medication App tracker. Download or scan the QR code to help you manage your PAH.
Supportive Treatments
Your healthcare provider may recommend supportive treatment to manage your PAH.
Managing Pulmonary Arterial Hypertension
Being diagnosed with a chronic illness like PAH is life-changing. It is critical to have a close relationship with a PAH expert and having a strong support system is also key to managing your disease. Your support system can include family members, friends, caregivers, other people diagnosed with PAH or support groups with members experiencing similar situations.
Many pulmonary hypertension specialists require visits every few months and regular testing such as echocardiograms and 6-minute walk testing. Your provider may also have you complete cardiopulmonary exercise testing (CPET), a specialized type of exercise test that measures your exercise ability. Some providers will do right heart catheterizations every year to see how well your 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.
Page last updated: June 5, 2026
