how pah is treated

PAH treatments work on 1 of 3 “pathways,” which means they each target 1 of these 3 imbalances. Because your doctor wants to find the right treatment or treatments for you, they may try different medications. In many cases, your doctor may offer more than 1 medicine to help treat the different imbalances at the root of your PAH.

Nitric oxide

How it works:
Slows the breakdown and/or enhances the production of your body's nitric oxide to keep the blood vessels in your lungs open


Types of medicines:

  • Phosphodiesterase type 5 (PDE-5) inhibitor

  • Soluble guanylate cyclase stimulator (sGCS)


Administration:

Oral therapy

Endothelin

How it works:
Helps prevent extra endothelin from closing or tightening the blood vessels in your lungs


Types of medicines:

  • Endothelin receptor antagonists (ERA)


Administration:

Oral therapy

Prostacyclin

How it works:
Mimics some of the effects of natural prostacyclin to help the blood vessels in your lungs stay open


Types of medicines:

  • Prostacyclin class therapies, including

    — Prostacyclin analogues

    — IP receptor agonists


Administration:

Oral, inhaled, and infused pump therapy

While there are 14 approved treatments for PAH, most of the treatments offered by United Therapeutics focus on the prostacyclin pathway and are considered prostacyclin analogues. Learn more about prostacyclin analogues.

Remember, PAH is a progressive disease, which means your body may have different treatment needs over time.

The medications that you start on at first may not be enough over the course of your disease. If you feel that your medications aren’t meeting your treatment goals or are no longer helping in the way they once did, talk to your doctor right away. See our Treatment Goals page for a list of goals you can set with your doctor.