Types of PAH Treatments

Connecting the pathways to your lungs

PAH is a chronic disease, but it can be treated with several medications. To understand how PAH is treated, it’s important to know what medications are available and why they might be needed.

Remember that PAH involves the narrowing of the blood vessels in your lungs. Why does this happen?

An imbalance of 3 natural substances

Small blood vessels in your lungs produce 3 natural substances: nitric oxide, endothelin, and prostacyclin.

Having the right amount of each of these 3 natural substances helps keep your blood vessels healthy. The right balance enables red blood cells to flow freely through the lungs and carry oxygen to the rest of the body.

When you have PAH, 1 or more of the 3 substances become out of balance. PAH medications work to impact that imbalance.

Currently, there is no test available to determine which of the 3 substances needs to be adjusted. Because patients with PAH generally respond better when more than 1 pathway is treated, it’s suspected that many patients have more than 1 pathway out of balance.

PAH medications help restore balance among the 3 substances

Each PAH medication works to impact only 1 of the 3 pathways: the nitric oxide pathway, the endothelin pathway, or the prostacyclin pathway. Each medication helps bring 1 substance back into balance.

Wanting to feel more like yourself?

PAH treatment guidelines recommend that most patients should have more than 1 pathway treated. Research shows that for many people with PAH, taking more than 1 medication to treat more than 1 pathway can delay disease progression.

An analysis of multiple studies involving more than 4,000 patients found that patients who took only 1 PAH medication were at a higher risk of their PAH worsening. So, if you’re taking 2 or 3 medications to treat your PAH, it’s more likely your PAH will remain the same or improve.

The analysis also found that taking at least 2 PAH medications helped

• Improve 6-minute walk distance
• Improve Functional Class

Adding a PAH medication could lead to additional symptom control and allow you to spend more time feeling like yourself. If you are still experiencing symptoms, talk with your healthcare provider about your treatment options. There may be more you can do.