PAH Medication & Treatment Guide

Medication Names: Tadalafil, Sildenafil

What It Does: Helps relax and widen blood vessels in the lungs, making it easier for blood to flow through them 

Common Side Effects: Headache, flushing (warmth and redness in the face, neck and upper chest), dizziness, upset stomach, runny or stuffy nose, muscle pain, back pain

Delivery Method: Oral

Additional Information: May improve ability to do physical activity and help slow disease from getting worse.

Medication Names: Riociguat (Adempas ®)

What It Does: Helps relax and widen blood vessels in the lungs, allowing blood to flow more easily.

Common Side Effects: Headache, indigestion, nausea, dizziness, low blood pressure, anemia, constipation.

*Note: Riociguat must not be used at the same time as PDE5 inhibitors (e.g. sildenafil or tadalafil) due to the risk of significant hypotension.

Delivery Method: Oral

Additional Information: May improve ability to do physical activity and help slow the disease from getting worse.

Medication Names: Bosentan, Ambrisentan, Macitentan

What It Does: Helps relax and widen blood vessels in the lungs, making it easier for blood to flow and reducing strain on the heart. 

Common Side Effects: Headache, swelling in ankles, feet, legs and abdomen, flushing (warmth and redness in the face, neck and upper chest), cold-like symptoms, unusual or rapid weight gain or weight loss, tingling of hands or feet.

*Note: Bosentan, ambrisentan and macitentan can cause harm to an unborn baby. Talk to your provider about potential risks and discuss family planning, including the need for effective birth control. 

Delivery Method: Oral

Additional Information: May improve ability to do physical activity and helps slow disease progression.

Medication Names: Epoprostenol (Flolan®, Veletri®), Treprostinil (Remodulin®, Orenitram®, Tyvaso®), Iloprost (Ventavis®), Tyvaso DPI (inhaled)

What It Does: Helps relax and widen blood vessels in the lungs, making it easier for blood to flow through them

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

Delivery Method: Oral, inhaled, intravenous (IV) or subcutaneous

Additional Information: May improve ability to do physical activity, reduce symptoms and help slow disease progression

Medication Names: Selexipag ((Uptravi®)

What It Does: Helps relax and widen blood vessels in the lungs.

Common Side Effects: Headache, jaw pain, muscle pain, joint pain, flushing, rash, anemia, nausea

Delivery Method: Oral

Additional Information: May slow disease from getting worse

Medication Names: Sotatercept-csrk (Winrevair)-Biologic

What It Does: Helps reduce the risk of PAH worsening events, including hospitalization and the need for transplantation, or death.

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

*Note: While taking this medication, you will need regular blood tests to monitor your blood cell and platelet levels and check for an increase in red blood cells. Your healthcare provider will want to monitor you closely to ensure this treatment remains safe for you.

Delivery Method: Subcutaneous (under-the-skin) injection

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

Medication Names: Opsynvi®

What It Does: Helps prevent narrowing and remodeling of blood vessels in the lungs Common Side Effects: Edema/fluid retention, anemia, and headache/migraine

Delivery Method: Oral

Additional Information: May help slow disease progression, reduce the risk of hospitalizations for PAH and  improve ability to do physical activity