Diagnostic delay in pulmonary arterial hypertension: Insights from the Australian and New Zealand pulmonary hypertension registry

Victor Khou¹, James J Anderson², Geoff Strange³, Carolyn Corrigan⁴, Nicholas Collins⁵, David S Celermajer^{1

6}, Nathan Dwyer⁷, John Feenstra⁸, Mark Horrigan⁹, Dominic Keating^{10

11}, Eugene Kotlyar^{4

12}, Melanie Lavender¹³, Tanya J McWilliams¹⁴, Peter Steele¹⁵, Robert Weintraub¹⁶, Helen Whitford^{10

11}, Ken Whyte¹⁴, Trevor J Williams^{10

11}, Jeremy P Wrobel^{3

13}, Anne Keogh^{4

12}, Edmund M Lau^{1

17}

Affiliations

¹ Sydney Medical School, University of Sydney, Sydney, NSW, Australia.
² Respiratory Department, Sunshine Coast University Hospital, Sunshine Coast Region, QLD, Australia.
³ School of Medicine, University of Notre Dame, Perth, WA, Australia.
⁴ Heart and Lung Transplant Unit, St Vincent's Hospital, Sydney, NSW, Australia.
⁵ Cardiovascular Department, John Hunter Hospital, Newcastle, NSW, Australia.
⁶ Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia.
⁷ Cardiology Department, Royal Hobart Hospital, Hobart, TAS, Australia.
⁸ Department of Thoracic Medicine, Prince Charles Hospital, Brisbane, QLD, Australia.
⁹ Department of Cardiology, Austin Health, Melbourne, VIC, Australia.
¹⁰ Department of Medicine, Monash University, Melbourne, VIC, Australia.
¹¹ Department Allergy Immunology and Respiratory Medicine, Alfred Hospital, Melbourne, VIC, Australia.
¹² Faculty of Medicine, University of New South Wales, Sydney, NSW, Australia.
¹³ Advanced Lung Disease Unit, Fiona Stanley Hospital, Perth, WA, Australia.
¹⁴ Greenlane Respiratory Services, Auckland City Hospital, Auckland, New Zealand.
¹⁵ Department of Cardiology, Royal Adelaide Hospital, Adelaide, SA, Australia.
¹⁶ Department of Cardiology, Royal Children's Hospital, Melbourne, VIC, Australia.
¹⁷ Department of Respiratory Medicine, Royal Prince Alfred Hospital, Sydney, NSW, Australia.

PMID: 31997504
DOI: 10.1111/resp.13768

Diagnostic delay in pulmonary arterial hypertension: Insights from the Australian and New Zealand pulmonary hypertension registry

Victor Khou et al. Respirology. 2020 Aug.

. 2020 Aug;25(8):863-871.

doi: 10.1111/resp.13768. Epub 2020 Jan 30.

Authors

Affiliations

¹ Sydney Medical School, University of Sydney, Sydney, NSW, Australia.
² Respiratory Department, Sunshine Coast University Hospital, Sunshine Coast Region, QLD, Australia.
³ School of Medicine, University of Notre Dame, Perth, WA, Australia.
⁴ Heart and Lung Transplant Unit, St Vincent's Hospital, Sydney, NSW, Australia.
⁵ Cardiovascular Department, John Hunter Hospital, Newcastle, NSW, Australia.
⁶ Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia.
⁷ Cardiology Department, Royal Hobart Hospital, Hobart, TAS, Australia.
⁸ Department of Thoracic Medicine, Prince Charles Hospital, Brisbane, QLD, Australia.
⁹ Department of Cardiology, Austin Health, Melbourne, VIC, Australia.
¹⁰ Department of Medicine, Monash University, Melbourne, VIC, Australia.
¹¹ Department Allergy Immunology and Respiratory Medicine, Alfred Hospital, Melbourne, VIC, Australia.
¹² Faculty of Medicine, University of New South Wales, Sydney, NSW, Australia.
¹³ Advanced Lung Disease Unit, Fiona Stanley Hospital, Perth, WA, Australia.
¹⁴ Greenlane Respiratory Services, Auckland City Hospital, Auckland, New Zealand.
¹⁵ Department of Cardiology, Royal Adelaide Hospital, Adelaide, SA, Australia.
¹⁶ Department of Cardiology, Royal Children's Hospital, Melbourne, VIC, Australia.
¹⁷ Department of Respiratory Medicine, Royal Prince Alfred Hospital, Sydney, NSW, Australia.

PMID: 31997504
DOI: 10.1111/resp.13768

Abstract

Background and objective: Early diagnosis of PAH is clinically challenging. Patterns of diagnostic delay in Australian and New Zealand PAH populations have not been explored in large-scale studies. We aimed to evaluate the magnitude, risk factors and survival impact of diagnostic delay in Australian and New Zealand PAH patients.

Methods: A cohort study of PAH patients from the PHSANZ Registry diagnosed from 2004 to 2017 was performed. Diagnostic interval was the time from symptom onset to diagnostic right heart catheterization as recorded in the registry. Factors associated with diagnostic delay were analysed in a multivariate logistic regression model. Survival rates were compared across patients based on the time to diagnosis using Kaplan-Meier method and Cox regression.

Results: A total of 2044 patients were included in analysis. At diagnosis, median age was 58 years (IQR: 43-69), female-to-male ratio was 2.8:1 and majority of patients were in NYHA FC III-IV (82%). Median diagnostic interval was 1.2 years (IQR: 0.6-2.7). Age, CHD-PAH, obstructive sleep apnoea and peripheral vascular disease were independently associated with diagnostic interval of ≥1 year. No improvement in diagnostic interval was seen during the study period. Longer diagnostic interval was associated with decreased 5-year survival.

Conclusion: PAH patients experience significant diagnostic interval, which has not improved despite increased community awareness. Age, cardiovascular and respiratory comorbidities are significantly associated with longer time to diagnosis. Mortality rates appear higher in patients who experience longer diagnostic interval.

Keywords: cohort studies; delayed diagnosis; mortality; pulmonary hypertension; survival analysis.

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Comment in

The 'great wait' for diagnosis in pulmonary arterial hypertension.
Weatherald J, Humbert M. Weatherald J, et al. Respirology. 2020 Aug;25(8):790-792. doi: 10.1111/resp.13814. Epub 2020 Apr 1. Respirology. 2020. PMID: 32239600 No abstract available.

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Diagnostic delay in pulmonary arterial hypertension: Insights from the Australian and New Zealand pulmonary hypertension registry

Affiliations

Diagnostic delay in pulmonary arterial hypertension: Insights from the Australian and New Zealand pulmonary hypertension registry

Authors

Affiliations

Abstract

Comment in

References

REFERENCES

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MeSH terms

Grants and funding

LinkOut - more resources

Full Text Sources

Other Literature Sources