PAH Treatment Guidelines

Use risk assessment to determine initial treatment plan and escalate therapy appropriately

PAH treatment guidelines provide a road map to managing your patients with PAH. With a risk assessment upon diagnosis, and then at least once every 3 to 6 months, you can adequately treat and assess your patients, escalating therapy to improve risk status as guidelines recommend.1

Aiming for low-risk status will give your patient a better likelihood of survival.1-5

2018 WSPH treatment algorithm1*



Low- or intermediate-risk patients

Treat with initial ERA+ PDE-5i combination therapy

High-risk patients

Treat with initial combination including IV prostacyclin-class therapy


Calculate risk status every 3 to 6 months


Add treatment if patients are not at low-risk status

*This chart is adapted from the treatment algorithm for nonvasoreactive patients with PAH confirmed by an expert center. Patients with idiopathic PAH, heritable PAH, or drug-induced PAH who are vasoreactive may initially receive calcium channel blocker therapy.Initial oral monotherapy has a residual role in the treatment of PAH.

Initial therapy

In addition to addressing general health and supportive care considerations, treatment guidelines require PAH-specific treatment based on risk status for patients with PAH.1

  • Low- and intermediate-risk patients should receive ERA + PDE-5i combination therapy at diagnosis. Monotherapy has a residual role in today's treatment guidelines
  • High-risk patients should receive combination therapy that includes an IV prostacyclin therapy.1 If your patient is high risk and in need of parenteral prostacyclin therapy, consider referral or consult to a PAH specialist for initiation

Monotherapy has a residual role as initial therapy1

In current PAH treatment guidelines, monotherapy is reserved for a small subset of patients with PAH. These include:

  • Patients treated with monotherapy for >5-10 years while maintaining a low-risk profile
  • Patients with very mild disease (WHO FC I, PVR 3-4 WU, mPAP <50 mm Hg, normal RV at Echo)
  • Patients for whom combination therapy is unavailable or contraindicated (eg, severe liver disease)

Hear from an expert

"Our treatment goal is really to get patients to low risk and then monitoring them continuously...always reassessing, always trying to drive them to this low-risk goal, because we know that that improves not only mortality.. but also makes it a much less morbid disease."

-Dr. Raymond Benza
The Ohio State University

Is it time to add another therapy to help your patients reach low-risk status?

Calculate Risk Now

Therapy escalation

Treating to low risk can improve your patient’s prognosis for survival. Because of this data, the 2018 WSPH treatment guidelines recommend that therapy be escalated if your patient has not met their low-risk goal.1 Differences in survival across risk status groups are evident early on in the course of the disease, and achieving low-risk status within 1 year of diagnosis leads to a better 5-year prognosis.2-5

Signs of initial improvement or general stability can be expected, but may not correlate with low-risk status. Remember, even FC II patients can be intermediate or high risk.6 That is one reason why formal risk calculation should be used. By conducting a formal risk calculation, you can identify when it’s time to escalate your patient’s treatment in accordance with today's treatment guidelines.1

Because patients are typically started on medications that treat the endothelin and/or nitric oxide pathways, treatment escalation to a prostacyclin-class therapy may be considered if patients are not at low-risk status after 3 to 6 months of initial therapy.1 If your patient is not taking a prostacyclin-class therapy and is not at low-risk status after their first 3 to 6 months of initial treatment, they may benefit from the addition of a prostacyclin-class medication.1,7

Hear from an expert

“I think pretty much every encounter we have with a patient, it’s an opportunity to assess their risk and consider whether or not we’ve gotten them into that low-risk status because that’s where we want them. So we can look forward and have confidence that they’re going to do well.”

-Dr. Vallerie McLaughlin
University of Michigan, Ann Arbor

Podcast icon
Click to expand transcript

Dr. Vallerie McLaughlin:

Let's talk about how often we should be performing those risk assessments. What's your approach to that?

Dr. Raymond Benza:

I think at least at baseline is probably one of the most important parts of risk stratification. Really, when a patient comes to your clinic for the first time, they may not have been on therapy. They're just newly diagnosed. They're really assessing their baseline risk; it’s very, very important. Not only for the treatments that we decide to place them on, but also for the patient's information and their own means to plan their lives for the next year or 2. And then after that initial assessment, and I think at minimum, every 3 to 6 months, we should be evaluating our patients, but I'd like to get your opinion on a more detailed approach and maybe peaking at a patient every time we see him in our clinic. What do you think about doing risk assessments even in our routine clinic?

Dr. Vallerie McLaughlin:

Oh, Ray. I think that's really key. I do that every single time I see a patient, I think pretty much every encounter we have with a patient, it's an opportunity to assess their risk and consider whether or not we've gotten them into that low-risk status because that's where we want them. So we can look forward and have confidence that they're going to do well.

Learn about treatment pathways

Treat Multiple Pathways
Echo=echocardiogram; ERA=endothelin receptor antagonist; FC=Functional Class; IV=intravenous; mPAP=mean pulmonary arterial pressure; PDE-5i=phosphodiesterase type 5 inhibitor; PVR=pulmonary vascular resistance; RV=right ventricle; WHO=World Health Organization; WSPH=World Symposium on Pulmonary Hypertension; WU=Wood unit.References: 1. Galiè N, et al. Eur Respir J. 2019;53(1):1801889. 2. Boucly A, et al. Eur Respir J. 2017;50(2):1700889. 3. Hoeper MM, et al. Eur Respir J. 2017;50(2):1700740. 4. Kylhammar D, et al. Eur Heart J. 2018;39(47):4175-4181. 5. Benza RL, et al. Chest. 2019;156(2):323-337. 6. Sahay S, et al. PLoS One. 2020;15(11):e0241504. 7. Barst RJ, et al. N Engl J Med. 1996;334(5):296-301.