From the Guidelines
Pulmonary hypertension treatments available on Australia's Pharmaceutical Benefits Scheme (PBS) include several medications that target different pathways of the disease, with the most recent and highest quality evidence supporting the use of endothelin receptor antagonists, phosphodiesterase 5 inhibitors, and prostacyclin analogues. The PBS subsidizes medications such as sildenafil (20mg three times daily) 1, bosentan (62.5mg twice daily initially, increasing to 125mg twice daily after 4 weeks) 1, ambrisentan (5-10mg once daily) 1, macitentan (10mg once daily) 1, and selexipag (starting at 200mcg twice daily with gradual titration) 1. For severe cases, epoprostenol and iloprost may be available through hospital-based programs.
Key Medications and Dosages
- Endothelin receptor antagonists:
- Phosphodiesterase 5 inhibitors:
- Prostacyclin analogues:
- Epoprostenol: 1–12ng/kg/min continuous intravenous infusion via central venous catheter 1
- Iloprost, inhaled: Initial dose: 2.5μg inhaled; if well-tolerated, then 5μg subsequent doses 6–9 times per day as needed; not more than once every 2h while awake 1
- Selexipag: 200 to 1600 μg twice per day orally 1
To access these medications through the PBS, patients must meet specific criteria including confirmation of pulmonary arterial hypertension through right heart catheterization, specific WHO functional classifications, and demonstration of vasoreactivity testing results. Treatment must be initiated by a specialist experienced in pulmonary hypertension management, typically a cardiologist or respiratory physician. Regular follow-up assessments are required to continue PBS coverage, usually every 6 months, to document treatment response. These medications work by dilating pulmonary blood vessels, inhibiting cell proliferation, and reducing pulmonary vascular resistance to improve exercise capacity and quality of life for patients with this progressive condition 1.
From the FDA Drug Label
Treprostinil is indicated for the treatment of pulmonary arterial hypertension (PAH; WHO Group 1) to diminish symptoms associated with exercise Ambrisentan tablets are indicated for the treatment of pulmonary arterial hypertension (PAH) (WHO Group 1): To improve exercise ability and delay clinical worsening
The treatment options for Pulmonary Hypertension (PH) listed are:
- Treprostinil (SQ): for the treatment of pulmonary arterial hypertension (PAH; WHO Group 1) to diminish symptoms associated with exercise 2
- Ambrisentan (PO): to improve exercise ability and delay clinical worsening in patients with PAH (WHO Group 1) 3 Note that the Pharmaceutical Benefits Scheme (PBS) in Australia is not mentioned in the provided drug labels.
From the Research
Treatment Options for Pulmonary Hypertension (PH) in Australia
The treatment options for Pulmonary Hypertension (PH) listed on the Pharmaceutical Benefits Scheme (PBS) in Australia include:
- Endothelin receptor antagonists, such as bosentan, ambrisentan, and macitentan, which are used to treat pulmonary arterial hypertension (PAH) 4, 5, 6
- Phosphodiesterase-5 inhibitors, which are used to treat PAH 4, 7
- Soluble guanylate cyclase stimulators, such as riociguat, which are used to treat PAH and chronic thromboembolic pulmonary hypertension (CTEPH) 4, 7
- Prostacyclin analogues and prostacyclin receptor agonists, which are used to treat PAH 4, 7
Availability of Treatment Options on the PBS
Studies have shown that bosentan, ambrisentan, and macitentan are available in Australia for the treatment of PAH and are listed on the PBS 5, 6. However, the availability of other treatment options, such as combination therapy, may be limited in Australia 8.
Efficacy of Treatment Options
Research has demonstrated that these treatment options can improve symptoms, exercise capacity, and quality of life for patients with PAH, as well as delay time to clinical worsening 4, 6, 7. Additionally, studies have shown that macitentan has higher treatment adherence compared to bosentan and ambrisentan in Australian patients with PAH 5.