Survival of idiopathic pulmonary arterial hypertension patients in the modern era in Australia and New Zealand

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

Research output: Contribution to journalArticlepeer-review

26 Citations (Scopus)


Background: Epidemiology 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. Methods: Consecutive cases of idiopathic, heritable and drug-induced PAH were prospectively enrolled into an Australian and New Zealand Registry. Results: Between January 2012 and December 2016, a total of 220 incident cases were enrolled (mean age 57.2. ±. 18.7. years, 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.
Original languageEnglish
Pages (from-to)1368-1375
Number of pages8
JournalHeart Lung and Circulation
Issue number11
Publication statusPublished - Nov 2018
Externally publishedYes


  • Pulmonary arterial hypertension
  • Pulmonary hypertension
  • Survival


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