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Title: Diagnostic delay in pulmonary arterial hypertension: Insights from the Australian and New Zealand pulmonary hypertension registry.
Austin Authors: Khou, Victor;Anderson, James J;Strange, Geoff;Corrigan, Carolyn;Collins, Nicholas;Celermajer, David S;Dwyer, Nathan;Feenstra, John;Horrigan, Mark ;Keating, Dominic;Kotlyar, Eugene;Lavender, Melanie;McWilliams, Tanya J;Steele, Peter;Weintraub, Robert;Whitford, Helen;Whyte, Ken;Williams, Trevor J;Wrobel, Jeremy P;Keogh, Anne;Lau, Edmund M
Affiliation: Respiratory Department, Sunshine Coast University Hospital, Sunshine Coast Region, QLD, Australia
Advanced Lung Disease Unit, Fiona Stanley Hospital, Perth, WA, Australia
Department of Respiratory Medicine, Royal Prince Alfred Hospital, Sydney, NSW, Australia
Department of Cardiology, Royal Children's Hospital, Melbourne, VIC, Australia
Department of Cardiology, Royal Adelaide Hospital, Adelaide, SA, Australia
Department of Thoracic Medicine, Prince Charles Hospital, Brisbane, QLD, Australia
Cardiology Department, Royal Hobart Hospital, Hobart, TAS, Australia
Cardiovascular Department, John Hunter Hospital, Newcastle, NSW, Australia
Sydney Medical School, University of Sydney, Sydney, NSW, Australia
Department of Cardiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia
Department of Medicine, Monash University, Melbourne, VIC, Australia
Department Allergy Immunology and Respiratory Medicine, Alfred Hospital, Melbourne, VIC, Australia
Heart and Lung Transplant Unit, St Vincent's Hospital, Sydney, NSW, Australia
Faculty of Medicine, University of New South Wales, Sydney, NSW, Australia
School of Medicine, University of Notre Dame, Perth, WA, Australia
Greenlane Respiratory Services, Auckland City Hospital, Auckland, New Zealand
Issue Date: Aug-2020 2020-01-30
Publication information: Respirology 2020; 25(8): 863-871
Abstract: 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. 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. 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. 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.
DOI: 10.1111/resp.13768
ORCID: 0000-0002-1890-6092
PubMed URL: 31997504
Type: Journal Article
Subjects: cohort studies
delayed diagnosis
pulmonary hypertension
survival analysis
Appears in Collections:Journal articles

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