Update on pharmacotherapy for pulmonary hypertension

Prior, David L; Adams, Heath; Williams, Trevor J

doi:10.5694/mja16.00468

Topics

Cardiovascular diseases

Summary

Pulmonary arterial hypertension (PAH) is a rare disease with a poor prognosis if not treated.
Pharmacological treatment options for PAH have increased significantly over the past 10 years, with availability of intravenous, oral and inhaled drugs targeting the nitric oxide, endothelin and prostacyclin pathways.
Treatment with these therapies in specialised pulmonary hypertension centres has resulted in reductions in patient symptoms, disease progression and mortality, and improved exercise capacity.
Recognition of chronic thromboembolic pulmonary hypertension is important, as this cause of pulmonary hypertension may be amenable to surgical treatment.
Several new oral drugs, including macitentan, riociguat and selexipag, some of which have novel modes of action, and the use of combinations of PAH drugs have recently been shown to be beneficial in treating PAH and are likely to change treatment for this condition in the future.

1 St Vincent's Hospital, Melbourne, VIC
2 Royal Hobart Hospital, Hobart, TAS
3 Alfred Hospital, Melbourne, VIC

Correspondence: david.prior@svha.org.au

Competing interests:

David Prior has received consultancy fees and travel assistance from Actelion Australia and travel assistance from GlaxoSmithKline. Trevor Williams is a member of the scientific advisory boards of Actelion Australia, GlaxoSmithKline and Bayer. He has received educational and research funding and travel assistance from Actelion Australia.

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