Chronic thromboembolic pulmonary hypertension in Australia and New Zealand: an analysis of the PHSANZ registry

Katherine Kearney, Joshua Gold, Carolyn Corrigan, Kumud Dhital, David Boshell, David Haydock, David McGiffin, Michael Wilson, Nicholas Collins, Rachael Cordina, Nathan Dwyer, John Feenstra, Melanie Lavender, Jeremy Wrobel, Helen Whitford, Trevor Williams, Dominic Keating, Ken Whyte, Tanya McWilliams, Anne KeoghGeoff Strange, Eugene Kotlyar, James Anderson, Edmund M. T. Lau*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

3 Citations (Scopus)

Abstract

Background and objective: Chronic thromboembolic pulmonary hypertension (CTEPH) is a serious condition occurring in 2%–4% of patients after acute pulmonary embolism. Pulmonary endarterectomy (PEA) is a potential cure for technically operable disease. The epidemiology and long-term outcomes of CTEPH have not been previously described in Australia and New Zealand. Methods: Data were extracted from the Pulmonary Hypertension Society of Australia and New Zealand (PHSANZ) registry for patients diagnosed with CTEPH between January 2004 and March 2020. Baseline characteristics, treatment strategies, outcome data and long-term survival are reported. Results: A total of 386 patients were included with 146 (37.8%) undergoing PEA and 240 (62.2%) in the non-PEA group. PEA patients were younger (55 ± 16 vs. 62 ± 16 years, p < 0.001) with higher baseline 6-min walk distance (6MWD; 405 ± 122 vs. 323 ± 146 m, p = 0.021), whilst both groups had similar baseline pulmonary haemodynamics. Pulmonary hypertension-specific therapy was used in 54% of patients post-PEA and 88% in the non-PEA group. The 1-, 3- and 5-year survival rates were 93%, 87% and 84% for the PEA group compared to 86%, 73% and 62%, respectively, for the non-PEA group (p < 0.001). Multivariate survival analysis showed baseline 6MWD was an independent predictor of survival in both operated and medically managed patients. Conclusion: In this first multicentre report of CTEPH in Australia and New Zealand, long-term survival is comparable to that in other contemporary CTEPH registries. However, PEA was only performed in a minority of CTEPH patients (37.8%) and significantly less than overseas reports. Greater awareness of PEA and improved patient access to experienced CTEPH centres are important priorities.

Original languageEnglish
Pages (from-to)1171-1180
Number of pages10
JournalRespirology
Volume26
Issue number12
DOIs
Publication statusPublished - Dec 2021

Keywords

  • chronic thromboembolic pulmonary hypertension
  • long-term survival
  • medical therapy
  • PHSANZ registry
  • pulmonary endarterectomy
  • pulmonary hypertension

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