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Respirology. 2021 Dec;26(12):1171-1180. doi: 10.1111/resp.14162. Epub 2021 Oct 05.

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

Respirology (Carlton, Vic.)

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 Keogh, Geoff Strange, Eugene Kotlyar, James Anderson, Edmund Mt Lau

Affiliations

  1. Heart and Lung Transplant Unit, St Vincent's Hospital Sydney, Sydney, New South Wales, Australia.
  2. University of New South Wales, Sydney, New South Wales, Australia.
  3. Liverpool Hospital, Sydney, New South Wales, Australia.
  4. Medical Imaging Department, St Vincent's Hospital Sydney, Sydney, New South Wales, Australia.
  5. Greenlane Cardiothoracic Surgical Unit, Auckland City Hospital, Auckland, New Zealand.
  6. Cardiothoracic Surgery, Alfred Hospital, Melbourne, Victoria, Australia.
  7. Macquarie University Hospital, Sydney, New South Wales, Australia.
  8. John Hunter Hospital, Newcastle, New South Wales, Australia.
  9. Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia.
  10. Sydney Medical School, University of Sydney, Camperdown, New South Wales, Australia.
  11. Cardiology Department, Royal Hobart Hospital, Hobart, Tasmania, Australia.
  12. Thoracic Medicine, The Prince Charles Hospital, Brisbane, Queensland, Australia.
  13. Advanced Lung Disease Unit, Fiona Stanley Hospital, Perth, Western Australia, Australia.
  14. School of Medicine, University of Notre Dame, Fremantle, Western Australia, Australia.
  15. Respiratory Medicine, Alfred Hospital, Melbourne, Victoria, Australia.
  16. Monash University, Melbourne, Victoria, Australia.
  17. Respiratory Medicine, Auckland City Hospital, Auckland, New Zealand.
  18. Respiratory Department, Sunshine Coast University Hospital, Birtinya, Queensland, Australia.
  19. Department of Respiratory and Sleep Medicine, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia.

PMID: 34608706 DOI: 10.1111/resp.14162

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.

© 2021 Asian Pacific Society of Respirology.

Keywords: PHSANZ registry; chronic thromboembolic pulmonary hypertension; long-term survival; medical therapy; pulmonary endarterectomy; pulmonary hypertension

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