• Geoff Strange, Edmund M Lau, Eleni Giannoulatou, Carolyn Corrigan, Eugene Kotlyar, Fiona Kermeen, Trevor Williams, David S Celermajer, Nathan Dwyer, Helen Whitford, Jeremy P Wrobel, John Feenstra, Melanie Lavender, Kenneth Whyte, Nicholas Collins, Peter Steele, Susanna Proudman, Vivek Thakkar, Dominic Keating, Anne Keogh, and PHSANZ Registry.
• School of Medicine, University of Notre Dame, Perth, WA, Australia; Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia; Pulmonary Hypertension Society of Australia and New Zealand, Sydney, NSW, Australia.
• Heart Lung Circ. 2018 Nov 1; 27 (11): 1368-1375.

BackgroundEpidemiology and treatment strategies continue to evolve in pulmonary arterial hypertension (PAH). We sought to define the characteristics and survival of patients with idiopathic, heritable and drug-induced PAH in the current management era.MethodsConsecutive cases of idiopathic, heritable and drug-induced PAH were prospectively enrolled into an Australian and New Zealand Registry.ResultsBetween January 2012 and December 2016, a total of 220 incident cases were enrolled (mean age 57.2±18.7years, female 69.5%) and followed for a median duration of 26 months (IQR17-39). Co-morbidities were common such as obesity (34.1%), systemic hypertension (30.5%), coronary artery disease (16.4%) and diabetes mellitus (19.5%). Initial combination therapy was used in 54 patients (dual, n=50; triple, n=4). Estimated survival rates at 1-year, 2-years and 3-years were 95.6% (CI 92.8-98.5%), 87.3% (CI 82.5-92.4%) and 77.0% (CI 70.3-84.3%), respectively. Multivariate analysis showed that male sex and lower 6-minute distance at diagnosis independently predicted worse survival, whereas obesity was associated with improved survival. Co-morbidities other than obesity did not impact survival. Initial dual oral combination therapy was associated with a trend towards better survival compared with initial oral monotherapy (adjusted HR=0.27, CI 0.06-1.18, p=0.082) CONCLUSIONS: The epidemiology and survival of patients with idiopathic PAH in Australia and New Zealand are similar to contemporary registries reported in Europe and North America. Male sex and poorer exercise capacity are predictive of mortality whereas obesity appears to exert a protective effect. Despite current therapies, PAH remains a life-threatening disease associated with significant early mortality.Copyright © 2017 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). All rights reserved.

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