What to Look Forward to in Pulmonary Arterial Hypertension Care


Though new therapies are still in the distant future, advances in diagnosis may be on the horizon.

In the last segment of their sit-down interview with MD Magazine® while at the 2018 CHEST Annual Meeting in San Antonio, TX, Gary Palmer, MD, MBA, Vice President of Medical Affairs for Actelion Pharmaceuticals and Victor Tapson, MD, Director of the Venous Thromboembolism & Pulmonary Vascular Disease Research Program at the Cedars-Sinai Medical Center, gave their final thoughts as to what future CHEST meetings may entail within regard to advances in pulmonary arterial hypertension (PAH) care.

MD Mag: How will pulmonary arterial hypertension care advance in the following years?

Tapson: I think one way it will look in the next few years—I don't think we're going to have a lot of dramatic changes in diagnostic testing. Echocardiology is always advancing to some degree. Right heart catheterization methods have been pretty standard for the past 25 years, really, not a lot has changed. I think suspecting it will be key, getting these tests, ruling out other causes pinning down what caused the PAH. Furthermore, is it idiopathic, scleroderma, liver disease, HIV, these kinds of things?

I think one of the biggest changes will be patients will be on more therapies, and maybe you start it early. We think about this sort of cancer analogy. In cancer patients there's aggressive induction therapy at first. You blast someone out of the water, you rest with therapy, and with PAH have intended to do that.

I've often thought it would make sense to put someone on intravenous prostacylcin right up front like we used to have to. I put them on Veletri right away titrate it up, see how they do, get them on some oral therapy, and maybe wean that off in 6 months or a year. Studies haven't been done. It's hard to convince people to go on parenteral therapy when they're doing pretty well. Now we have some pretty darn good oral therapies, but again, the triple therapy approach I think is going to be a big one, and maybe even drugs with new mechanisms.

Right now, we're treating the endothelin receptor pathway, across the PDE5 inhibitor pathway. But the cancer analogy: patients with cancer have anaerobic glycolysis, why is that? Well, there's analogies of PAH—the same thing might occur. Should we be using dichloroacetate or one of these drugs? Epigenetics are huge now. Things like histone modification, micro-RNAs, exploring these things doing different kinds of omics—metabolomics, proteomics—and deeper dives into phenotyping. I think they would be really important in the future, too.

Palmer: We’re continuing to invest heavily in this area. There's a lot of interest in it, we've made a lot of progress, but we have a long way to go still. I agree, I think we'll see more and more people on combination therapies in the future. But we continue to look for new targets, new opportunities.

Sadly, there's nothing in the real near-term future, but hopefully over the next 4 or 5 years we'll start to see some new opportunities and options coming forward. Similarly on the diagnostic side, we continue to look for biomarkers for things that we can use to identify the disease earlier and track it. And there's a lot of efforts ongoing to identify things that it may help in the diagnosis of disease.

Tapson: There's been interest in implantable monitors thatcan measure your PA pressure of moment-to-moment. I’m personally not sure we need that. I think we could do a pretty good job without that kind of thing. But as Gary mentioned, we may not have we have a lot of changes in how we do echos or right heart cath’s, and maybe we'll get better non-invasive diagnostic testing for a stroke and cardiac output, things like that, with a finger probe.

But again, I think the key thing is this diving in the phenotype. And right now, phenotypes are just general PAH, is it idiopathic or scleroderma. Knowing much more about genes, I think, will help us advance the field.

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