(PAH-Specific Medications)
These medications help open the blood vessels in the lungs so blood can move more easily. They are commonly used for pulmonary arterial hypertension (PAH).
Endothelin Receptor Antagonists (ERAs)
These medicines block endothelin, a chemical that makes lung blood vessels tighten.
They can:
- Relax and open narrowed blood vessels
- Lower pressure inside the lungs
- Improve breathing and activity levels
Examples: bosentan, ambrisentan, macitentan
PDE-5 Inhibitors / sGC Stimulator (Riociguat)
These medicines help the lungs use a natural chemical called nitric oxide, which relaxes blood vessels.
They can:
- Help lung blood vessels widen
- Improve blood flow
- Support exercise ability and reduce symptoms
Examples:
- PDE-5 Inhibitors: sildenafil, tadalafil
- sGC Stimulator: riociguat
Prostacyclin Pathway Therapies (Prostanoids)
These treatments replace or boost prostacyclin, a natural substance that helps keep lung blood vessels open and reduces inflammation.
They can:
- Strongly relax lung arteries
- Improve blood flow
- Reduce pressure on the right side of the heart
- Improve ability to be active
They come in different forms:
- IV or subcutaneous infusion: epoprostenol, treprostinil
- Inhaled therapies: iloprost, inhaled treprostinil
- Oral medications: selexipag, oral treprostinil
Your PH specialist will choose the form that fits your needs and lifestyle.
Activin Signaling Inhibitor (Sotatercept)
Sotatercept is a newer treatment that works by blocking signals that cause lung blood vessels to thicken and tighten. This helps rebalance how cells grow inside the vessels.
It can:
- Lower pressure in the lungs
- Improve how far people can walk
- Reduce strain on the heart
- Work well when added to other PAH medications
Most people who take sotatercept use it along with other PAH medicines.