The 3 most important recent advances in the management of pulmonary hypertension

Dr Adam Torbicki is a professor of medicine in the Center of Postgraduate Medical Education in Warsaw, Poland, past vice-president of the European Society of Cardiology, and coauthor of European guidelines on pulmonary hypertension.

What are the 3 most important recent advances in the management of pulmonary hypertension?

Prof. Adam Torbicki, MD, PhD: The 3 most important recent advances in pulmonary hypertension (PH)… I would start with a new drug that has been developed and is now already approved, and in many countries also reimbursed, which is an oral prostacyclin receptor agonist. So far we have been able only to use prostacyclin in a continuous infusion, usually to a large vein, after inserting a Hickman catheter—a very complex procedure. Sometimes we use infusions to subcutaneous tissue, just like in the case, for example, of insulin. This is also cumbersome for the patient. Also, prostacyclin derivatives were administered via inhalation, but then you need to inhale many times a day, and it is also not very easy and certainly affects quality of life. Now we have a new drug, which is simply administered orally and has a similar action. Probably [it] is not as effective as parenteral application of prostanoids, but it can serve as a kind of bridge to this most effective but also not very easy to apply therapy. So, this would be the first important recent advance.

Then we have something that is very thrilling and not very often used, and not very often needed, but promises a lot for the near future. This is the gene that is abnormal, mutated in the pulmonary veno-occlusive disease (PVOD). PVOD is one of the most difficult differential diagnoses in patients with PH. And now we have a mutation that causes PVOD and we don’t need to have any more diagnostic doubts regarding those patients in whom clinically we see severe PH, but we can detect the mutation of this new gene, because this makes our management strategy clearer. It is not very easy, this management strategy, often it will lead to lung transplant, but at least we are certain that we made the right decision.

And finally, a very recent and very important change that should affect everyday practice in PH is the new definition and the new cutoff level, above which we diagnose or we are expected to diagnose PH. It is no longer 25 mm Hg but it is 20. So we are going down. We are lowering the threshold closer to the normal values that are found in healthy people. As you probably remember, the mean normal value for mean pulmonary artery pressure (mPAP) is ~14 mm Hg, and >20 mm Hg it’s already abnormal. Now the new definition tries to adjust to these values. This is a bit problematic because we still do not have drugs that have been verified in this zone, which so far has been considered normal or near normal. So, we have a lot of things to do and a lot of trials to perform before we will be sure what to do with these patients. But we have to remember that the definition of PH has been changed.