PAH Medication & Treatment Guide

Learn more about the different types of medications that can help manage your PAH symptoms.

There are a variety of medicines available to treat your pulmonary arterial hypertension. 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. The medications you take may be pills, inhaled, or subcutaneous meaning you receive a shot of the medicine delivered through a small continuous pump.  It is important to work with your PAH specialist, a lung doctor or a heart doctor with expertise in this disease, to determine the treatment plan that will work best for you.

Types of Medications to Manage PAH

Select a medication pathway below to learn more about what each medication does, common side effects and additional information. A printable version of the Medication Guide is available to download.

Nitric Oxides

Helps blood vessels stay open and ensures blood is flowing properly

Medication Names: Tadalafil, Sidenafil

What It Does: Allows the lungs to relax and the blood vessels to expand, making it easier for your heart to pump blood through your body

Common Side Effects: Headache, flushing, lightheaded, nausea, runny or stuffy nose, muscle pain, back pain

Delivery Method: Oral, Subcutaneous (short-term only for subcutaneous)

Additional Information: Slows disease from getting worse, improves ability to do physical activity

Medication Names: Adempas, Riociguat 

What It Does: Allows the blood vessels in the lungs to relax helping more blood to flow through

Common Side Effects: Headache, indigestion, gastritis, dizziness, low blood pressure, anemia GERD, constipation, bleeding

Delivery Method: Oral

Additional Information: Slows disease from getting worse, improves ability to do physical activity

Endothelin Receptor Antagonists (ERAs)

Prevents blood vessels from narrowing

Medication Names: Bosentan, Ambrisentan, Macitentan

What It Does: Lowers blood pressure in the lungs, helping heart to pump blood more efficiently

Common Side Effects: Headache, joint pain, low blood pressure, fainting, flushing, irregular heartbeat, cold symptoms

Delivery Method: Oral

Additional Information: Improves ability to do physical activity, slows disease from getting worse, relieves symptoms

Prostaglandins / Prostacyclins

Helps blood vessels stay open

Medication Names: Epoprostenol, Flolan, Iloprost, Orenitram, Treprostinil, Treprostinil Inhaled, Remodulin, Tyvaso, Veletri, Ventavis

What It Does:  Allows the blood vessels in the lungs to relax; inhaled treatments relieve shortness of breath

Common Side Effects: Flushing, headache, muscle pain, cough, diarrhea, nausea, vomiting, indigestion, throat irritation, flu-like symptoms, rash

Delivery Method: Oral, inhaled, IV or subcutaneous

Additional Information: Improves ability to do physical activity

Medication Names: Selexipag

What It Does: Allows the blood vessels in the lungs to relax

Common Side Effects: Headache, jaw pain, muscle pain, joint pain, flushing, rash, anemia, nausea, pain/redness at injection site if injected

Delivery Method: Oral, subcutaneous

Additional Information: Slows disease from getting worse

Medication Names: Tyvaso DPI (Inhaled)

What It Does: Relaxes blood vessels, helping to widen the narrowed blood vessels in the lungs for better blood flow

Common Side Effects: Cough, headache, throat irritation, nausea, flushing, dizziness

Delivery Method: Inhaled

Additional Information: Improves ability to do physical activity, slows worsening of symptoms

Biologics

Helps keep the walls of the blood vessels from thickening

Medication Names: Sotatercept-csrk (Winrevair)

What It Does: Works to correct imbalances that impact the structure of your blood vessels

Common Side Effects: Headache, nosebleed, rash, tiny blood vessels that look pink or red on the skin, diarrhea, dizziness, redness

Delivery Method: Subcutaneous

Additional Information: Improves ability to do physical activity, reduces the risk of physical condition and symptoms getting worsening

Combination Medications

Helps blood vessels stay open

Medication Names: Opsynvi

What It Does: Counteracts the thickening of the arterial wall and organ damage; promotes muscle relaxation and widening of blood vessels

Common Side Effects: Edema/fluid retention, anemia, and headache/migraine

Delivery Method: Oral

Additional Information: Slows disease from getting worse and decreases risk of hospitalizations, improves ability to do physical activity

Talk to an Expert

Our Lung HelpLine is staffed by registered nurses and respiratory therapists ready to help answer your questions about PAH.
Contact HelpLine

Manage Your Medications

Use this tracker to manage what medications you are taking, what the medication is for, possible side effects, and more.
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Page last updated: September 13, 2024

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