A post-market review of medicines for pulmonary arterial hypertension (PAH) has effectively brought PBS listings up to date and consistent with the evidence as well as best international practice.

Effective from December 2022, the PBS subsidised access to treatment includes a combination of IV epoprostenol or inhaled iloprost and an endothelin receptor antagonist (ERA) as the first treatment option for patients with PAH WHO Functional Class IV symptoms and as a second treatment choice for patients with Functional Class III symptoms.

The PBS also extended subsidised access to triple therapy with an ERA plus epoprostenol or iloprost and a PDE-5 inhibitor for patients with Functional Class IV symptoms.

The long-awaited changes align patient access to PAH medicines more closely with clinical guidelines.

Respiratory physician Associate Professor Edmund Lau, from the Royal Prince Alfred Hospital in Sydney, told the limbic the important change was moving patients with severe disease beyond triple oral therapy to triple therapy including a parenteral prostacyclin.

“We are essentially up to date with the combinations that we would use for best clinical practice,” he said.

“So before, in the past maybe year a bit, we could get triple oral therapy with selexipag as the IP receptor agonist and then either ambrisentan or macitentan as the ERA and then sildenafil or taqdalafil as the PDE-5i.”

“But we would not actually be reimbursed for triple therapy with a parenteral prostacyclin which is essentially IV epoprostenol.”

He said epoprostenol and selexipag were completely different drugs.

“So now we can actually put people on IV epoprostenol plus an ERA plus a PDE-5i and that would be considered I guess the standard of care for people with the most severe disease that are eligible for aggressive treatment.”

Associate Professor Lau said a small proportion of patients would actually start triple therapy with a parenteral agent almost straightaway.

“In these patients where they are very, very sick and we want to actually be super aggressive in the way we treat them then yes, we will put them on all three drugs right from the start. It doesn’t need to be three drugs on day one…but over two or three weeks.”