Pulmonary Arterial Hypertension treatment options rapidly evolve (2)

Pulmonary Arterial Hypertension treatment options rapidly evolve (2)


– What is new in treatment of pulmonary arterial
hypertension? Dr. Anton Titov MD – So the treatment of pulmonary arterial hypertension
has come a long way since I got involved. When I first got involved, there was only
one drug, well, two drugs – there were calcium channel blockers, which are pretty ineffective
for the vast majority of patients with pulmonary arterial hypertension, and epoprostenol, which
was called Flolan at the time, which is an intravenous therapy. Prostacyclin was the other drug. And it really had a big impact on patients,
but it was a very complicated therapy that involved intravenous continuous infusion,
external pumps, and Hickman catheters. Over the course of the past 20 years the focus
in pulmonary arterial hypertension has been on three main pathways: endothelin pathway,
nitric oxide pathway, and prostacyclin pathway. So there been a number of drugs developed
– both intravenous and oral, and even inhaled, but none of them are home runs. They’re all effective in pulmonary arterial
hypertension to a certain extent. They’ve had a tremendous impact on survival
and functional status, quality of life. But there’s still tremendous room for much
more development [of pulmonary arterial hypertension therapy]. More and more the focus is now on metabolic
modulators, things that change mitochondrial function, things that are antiproliferative,
even some chemotherapeutic [medications] repurposing going on, and also anti-inflammatory targets,
in a complicated way. So I think we’re moving much more into the
realm where we are starting to treat pulmonary arterial hypertension like a metabolic neoplastic
disease more than a vasoconstrictor disease, which is what most of the focus has been on. So for somebody who has been diagnosed with
pulmonary arterial hypertension and the diagnosis has been established, are there stages of
therapy or lines of therapy, similar to the neoplastic disease treatment? What is a typical progression of treatment
for a pulmonary arterial hypertension patient? – So we grade patient severity of disease
much like we do in heart failure. And we use the WHO functional class approach. And really over the past two to three years
what’s become the standard of care for pulmonary arterial hypertension is using combination
therapy, just like we would for most any other complicated disease. These drugs for pulmonary arterial hypertension
tend to be quite expensive, so there’s been a reluctance over the past years purely because
of expense. Now we have clinical trial data that show
clear benefit in pulmonary arterial hypertension of using a combination therapy. So, depending on how sick a patient might
be – and by how sick I mean how short of breath,
how limited are they? Are they having issues with heart failure
at the time? We might start out with just oral therapy
and using a combination of two drugs. Usually it’s going to be a phosphodiesterase
5 inhibitor in combination with an endothelin antagonist. As patients with pulmonary arterial hypertension
progress, then we start to think more and more about prostacyclins. But the truth is that prostacyclins still
are our best therapies and probably if we use them earlier in disease, we’d get even
better results. So it’s a moving target how we treat patients. But what’s good is that it’s becoming a more
complex targeting of disease or at least a therapeutic approach to pulmonary arterial
hypertension, which I think is having better outcomes.

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