Pharmacological Treatment of Pulmonary Arterial Hypertension in Australia: Current Trends and Challenges

James Anderson; Melanie Lavender; Edmund Lau; David Celermajer; Nicholas Collins; Nathan Dwyer; John Feenstra; Mark Horrigan; Dominic Keating; Anne Keogh; Eugene Kotlyar; Benjamin Ng; Susanna Proudman; Peter Steele; Vivek Thakkar; Robert Weintraub; Helen Whitford; Trevor Williams; Jeremy Wrobel; Geoff Strange

doi:10.1016/j.hlc.2020.01.017

Pharmacological Treatment of Pulmonary Arterial Hypertension in Australia: Current Trends and Challenges

Heart Lung Circ. 2020 Oct;29(10):1459-1468. doi: 10.1016/j.hlc.2020.01.017. Epub 2020 Mar 6.

Authors

James Anderson¹, Melanie Lavender², Edmund Lau³, David Celermajer⁴, Nicholas Collins⁵, Nathan Dwyer⁶, John Feenstra⁷, Mark Horrigan⁸, Dominic Keating⁹, Anne Keogh¹⁰, Eugene Kotlyar¹⁰, Benjamin Ng¹¹, Susanna Proudman¹², Peter Steele¹³, Vivek Thakkar¹⁴, Robert Weintraub¹⁵, Helen Whitford⁹, Trevor Williams⁹, Jeremy Wrobel¹⁶, Geoff Strange¹⁷

Affiliations

¹ Advanced Lung Disease Unit, Fiona Stanley Hospital, Perth, WA, Australia; Respiratory Department, Sunshine Coast University Hospital, Birtinya, Qld, Australia. Electronic address: james.anderson2@health.qld.gov.au.
² Advanced Lung Disease Unit, Fiona Stanley Hospital, Perth, WA, Australia.
³ Department of Respiratory Medicine, Royal Prince Alfred Hospital, Sydney, NSW, Australia; Sydney Medical School, University of Sydney, Camperdown, NSW, Australia.
⁴ Sydney Medical School, University of Sydney, Camperdown, NSW, Australia; Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia.
⁵ John Hunter Hospital, Newcastle, NSW, Australia.
⁶ Cardiology Department, Royal Hobart Hospital, Hobart, Tas, Australia.
⁷ Thoracic Medicine, The Prince Charles Hospital, Brisbane, Qld, Australia.
⁸ The Austin Hospital, Melbourne, Vic, Australia.
⁹ Respiratory Department, Alfred Hospital, Melbourne, Vic, Australia; Monash University, Melbourne, Vic, Australia.
¹⁰ Heart and Lung Transplant Unit and Cardiology Department, St Vincent's Hospital, Sydney, NSW, Australia; University of New South Wales, Sydney, NSW, Australia.
¹¹ Nepean Hospital, Sydney, NSW, Australia.
¹² Rheumatology Unit, Royal Adelaide Hospital, Adelaide, SA, Australia.
¹³ Department of Cardiovascular Services, Royal Adelaide Hospital, Adelaide, SA, Australia.
¹⁴ Macquarie University, Department of Clinical Medicine, Macquarie Park, NSW, Australia; Department of Rheumatology, Liverpool Hospital, Liverpool, NSW, Australia.
¹⁵ Royal Children's Hospital, Melbourne, Vic, Australia; Murdoch Children's Research Institute, Melbourne, Vic, Australia; University of Melbourne, Melbourne, Vic, Australia.
¹⁶ Advanced Lung Disease Unit, Fiona Stanley Hospital, Perth, WA, Australia; University of Notre Dame, Perth, WA, Australia.
¹⁷ University of Notre Dame, Perth, WA, Australia.

PMID: 32280014
DOI: 10.1016/j.hlc.2020.01.017

Abstract

Background: Combination drug therapy for pulmonary arterial hypertension (PAH) is the international standard of care for most patients, however in Australia there are barriers to drug access. This study evaluates current treatment of PAH patients in Australia and the consistency of therapy with international guidelines.

Methods: Cross-sectional analysis of patients with Group 1 PAH enrolled in the Pulmonary Hypertension Society of Australia and New Zealand Registry (PHSANZ) at 31 December 2017. Drug treatment was classified as monotherapy or combination therapy and adequacy of treatment was determined by risk status assessment using the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) 2.0 risk calculator. Predictors of monotherapy were assessed using a generalised linear model with Poisson distribution and logarithmic link function.

Results: 1,046 patients met the criteria for analysis. Treatment was classified as monotherapy in 536 (51%) and combination therapy in 510 (49%) cases. Based on REVEAL 2.0, 184 (34%) patients on monotherapy failed to meet low-risk criteria and should be considered inadequately treated. Independent predictors of monotherapy included age greater than 60 years (risk ratio [RR] 1.23, 95% confidence interval [CI] 1.09-1.38; p=0.001), prevalent enrolment in the registry (RR 1.21 [95%CI 1.08-1.36]; p=0.001) and comorbid systemic hypertension (RR 1.17 [95%CI 1.03-1.32]; p=0.014), while idiopathic/heritable/drug-induced PAH subtype (RR 0.85 [95%CI 0.76-0.96]; p=0.006), functional class IV (RR 0.50 [95%CI 0.29-0.86]; p=0.012), increased right ventricular systolic pressure (RR 0.99 [95%CI 0.99-1.00]; p<0.001) and increased pulmonary vascular resistance (RR 0.96 [95%CI 0.95-0.98]; p<0.001) were less likely to be associated with monotherapy.

Conclusions: Most Australian PAH patients are treated with monotherapy and a significant proportion remain at risk of poor outcomes. This is below the standard of care recommended by international guidelines and at risk patients should be escalated to combination therapy.

Keywords: PAH; Pulmonary hypertension; Vasodilator.

Publication types

Multicenter Study

MeSH terms

Adult
Aged
Antihypertensive Agents / administration & dosage*
Australia / epidemiology
Cross-Sectional Studies
Dose-Response Relationship, Drug
Drug Therapy, Combination
Female
Humans
Male
Middle Aged
Pulmonary Arterial Hypertension / drug therapy*
Pulmonary Arterial Hypertension / epidemiology
Registries*
Young Adult

Substances

Antihypertensive Agents