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
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Article info
Publication history
Published online: March 06, 2020
Accepted:
January 31,
2020
Received in revised form:
December 25,
2019
Received:
August 28,
2019
Footnotes
Guarantor statement: James Anderson takes responsibility for the content of the manuscript, including the data and analysis.
Identification
Copyright
Crown Copyright © 2020 Published by Elsevier B.V. on behalf of Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). All rights reserved.
