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Pulmonary Arterial Hypertension
Expert Answers to Questions on Pulmonary Arterial Hypertension

Released: October 03, 2024

Expiration: October 02, 2025

Vallerie V. McLaughlin
Vallerie V. McLaughlin, MD
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Key Takeaways
  • New and emerging PAH therapies will offer patients additional options for managing their disease.

The following is a recap of key questions asked by healthcare professionals during a program featuring Vallerie V. McLaughlin, MD, discussing pulmonary arterial hypertension (PAH), including epidemiology and classification, diagnostic evaluation, guideline-based management, and emerging therapeutic agents.

Can one simultaneously treat idiopathic pulmonary fibrosis (IPF) and PAH (that is not from left-sided heart disease)?

This is a common problem that we see referred to cardiology from pulmonology. If a patient has IPF and PAH without left heart disease (normal wedge pressure), they are generally characterized as Group 3 PAH, meaning that their pulmonary hypertension is a result of their IPF. The one agent that is approved by the FDA for the treatment of pulmonary hypertension in the setting of IPF is inhaled treprostinil, which can be given concomitantly with some of the newer agents for PAH.

What new agents for PAH are you most excited about?

Sotatercept is new and is easy to be excited about. The STELLAR trial results were very impressive, where not only was the primary endpoint met, but also 8 of 9 secondary endpoints were met.

The drug development program for sotatercept includes trials studying sotatercept across the spectrum of patients with PAH. Sotatercept is being studied in patients who are typically too sick for enrollment on to trials. The results from the ZENITH trial, which studied sotatercept in patients with PAH with WHO functional class III or IV disease at high risk of mortality, should be published sometime in the next year. In addition, the HYPERION study of sotatercept in newly diagnosed patients with intermediate-risk to high-risk PAH will provide insights into using sotatercept earlier in the course of PAH.

Data from these studies will complement what we currently know about sotatercept use across the spectrum of patients with PAH.

Although I am excited about sotatercept, my excitement extends to other drugs in the development pipeline. Seralutinib is a drug being studied in phase III trials after demonstrating impressive results in phase II trials. The TORREY study demonstrated a significant reduction in pulmonary vascular resistance. Seeing the clinical effects of that improvement via the 6-minute walk test will be important.

Where do you think sotatercept will fit in the PAH guidelines?

It is important to understand the population in whom sotatercept use has been studied: patients with PAH who are still symptomatic despite receiving standard-of-care therapy. Sotatercept has not been studied as an initial therapy for patients with PAH. I think for most patients, dual oral therapy with an endothelin receptor antagonist (ERA) and a phosphodiesterase type 5 (PDE5) inhibitor will remain the first step. But then I see sotatercept potentially being used in patients who do not meet the criteria for low risk and still have room for improvement after receiving an ERA plus PDE5 inhibitor.

Will patients be able to stop other PAH therapies with the addition of sotatercept?

The therapy that patients want to get off of most commonly is the pump therapy—no one wants to carry a pump around with them. But sotatercept was not studied as a replacement for a patient’s current therapy. Instead, it was studied as add-on therapy. Having said that, there were perhaps a dozen or so patients who were able to come off of their pumps after starting sotatercept.

Stopping therapy is not something that I promise patients when we start them on sotatercept. But there may be select patients who have had near normalization of their hemodynamics on a prostacyclin that we think we can start them on sotatercept, see how they respond, and perhaps wean their prostacyclin down very carefully. This type of experience in select patients being cared for in very experienced centers might inform the best approach.

Your Thoughts?
What new or emerging treatment for patients with PAH are you most excited about? Join the discussion by posting a comment.

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