Survival of Idiopathic Pulmonary Arterial Hypertension Patients in the Modern Era in Australia and New Zealand

Strange, Geoff; Lau, Edmund; Giannoulatou, Eleni; Corrigan, Carolyn; Kotlyar, Eugene; Kermeen, F.; Williams, Trevor; Celermajer, David S.; Dwyer, Nathan; Whitford, Helen; Wrobel, Jérémy; Feenstra, John; Lavender, Melanie; Whyte, K.; Collins, Nicholas; Steele, Peter; Proudman, Susanna; Thakkar, Vivek; Keating, D.; Keogh, Anne

doi:10.1016/j.hlc.2017.08.018

Cited by 35 publications

(35 citation statements)

References 27 publications

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“…The single-centre Giessen PH Registry also reported similar, long-term transplant-free survival data from more than 2000 patients with different PH subtypes [52]. Moreover, the epidemiology and survival of patients with IPAH in Australia and New Zealand are similar to contemporary registries reported in Europe and North America [54]. In conclusion, there is an improving trend in the survival of PAH attributable to drug therapies, but the overall outcome of PAH still remains poor.…”

Section: Long-term Survival/overall Survivalmentioning

confidence: 53%

Learning from registries in pulmonary arterial hypertension: pitfalls and recommendations

Swinnen

Quarck

Godinas³

et al. 2019

Eur Respir Rev

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Pulmonary arterial hypertension is a rare and incurable chronic disease characterised by a progressive increase in pulmonary vascular resistance and right heart failure. Patient registries collecting observational data can be of great value in the understanding of clinical problems. While clinical trials provide data in selected patient populations, registries better depict real-life practice. This review aims to reflect the input of patient registries in the general knowledge of the disease. Advances in epidemiology of the different subgroups, including data on incidence and/or prevalence, increasing age at presentation and stagnating diagnostic delay are reported. The importance of haemodynamic definition criteria and cardiac comorbidities are underscored. The review also shows the major transformation that pulmonary arterial hypertension therapeutic management has undergone, with still insufficient use of combination therapies; consecutive improvement in outcome; upcoming evidence in disfavour of anticoagulation; and validity of the available risk-stratification tools derived from large registries. Product registries are also briefly presented. Finally, the benefits of registries and methodological aspects are discussed, including immortal time bias, registry data quality and recommendations from EU organisations (EUCERD and PARENT).

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Section: Long-term Survival/overall Survivalmentioning

confidence: 53%

Learning from registries in pulmonary arterial hypertension: pitfalls and recommendations

Swinnen

Quarck

Godinas³

et al. 2019

Eur Respir Rev

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“…These results are highly consistent with our findings, and we believe that mortality closely related to the WHO functional class. However, Strange's team believed that the epidemiology and survival of patients with idiopathic PAH in Australia and New Zealand were similar to contemporary registries reported in Europe and North America …”

Section: Discussionmentioning

confidence: 71%

“…Pulmonary hypertension is a major global health issue; it affects approximately 1% of the total population and increases up to 10% among individuals aged >65 years . Despite impressive advancements in elucidation of the pathobiology of PH and the introduction of targeted medical therapies, PH remains a life‐threatening disease with a poor prognosis, with estimated survival rates at 1 year, 2 years and 3 years of 95.6%, 87.3% and 77.0%, respectively, in Europe and North America . Thus, it is important to identify novel prognostic therapeutic targets and precise prognostic markers for this type of lung disease.…”

Section: Introductionmentioning

confidence: 99%

Serum cardiac troponin elevation predicts mortality in patients with pulmonary hypertension: A meta‐analysis of eight cohort studies

Yang

Zhang

et al. 2019

Clinical Respiratory J

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are first co-authors.A bbreviations: 95%CI, 95% confidence interval; CTEPH, chronic thromboembolic pulmonary hypertension; CTn, cardiac troponin; HR, hazard ratio; IPAH, idiopathic pulmonary arterial hypertension; NOS, Newcastle-Ottawa Scale; PAH, pulmonary arterial hypertension; PH, pulmonary hypertension. Correspondence AbstractObjective: To determine the association of serum cardiac troponin (cTn) with the mortality of pulmonary hypertension (PH) patients via a meta-analysis. Date Source: We searched PubMed and EMBASE from inception to October 25, 2017.Study Selection: The reference lists of the retrieved articles were also consulted.The Q test and I 2 test were used for to assess heterogeneity. The relationship between cTn elevation and mortality was analysed. Studies were stratified according to type of troponin (cTnT vs cTnI), region (Europe vs America) and follow-up length (≤3 years vs >3 years). Results: Eight studies with 739 patients were included in the meta-analysis. Cardiac troponin elevation ranged from 14.3% to 94.5%. Overall, 48.8% (39/80) of patients with elevated cTn died compared to 18.6% (45/242) of patients with normal cTn levels. These findings showed cTn elevation was significantly related to an increased mortality risk in PH patients [hazard ratio (HR) = 3.05, 95% confidence interval (95% CI) = 2.16-4.32, I 2 = 24.9%]. cTnI was better at predicting mortality than cTnT (HR = 3.37, 95%CI = 2.05-5.55 vs HR = 2.80, 95%CI = 1.97-3.98, respectively).American populations had increased mortality compared to European populations (HR = 4.23, 95%CI = 2.29-7.80 vs HR = 2.70, 95% CI = 1.95-3.74, respectively). How to cite this article: Xu S-L, Yang J, Zhang C-F, et al. Serum cardiac troponin elevation predicts mortality in patients with pulmonary hypertension: A meta-analysis of eight cohort studies. Clin Respir J. 2019;13:82-91. https://doi.

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“…Concurrent with this increased attention, a number of national and international registries have been established to assess the outcomes for patients in the ‘real world’. Such registries include the REVEAL registry in the USA, PHSANZ in Australia and New Zealand and several European registries . A recent publication in Respirology by Lim et al .…”

mentioning

confidence: 99%

“…Other contemporary registries report slightly better survival rates. Published data from the Australian/NZ registry reported the most recent incident cohort (2012–2016) having a 3‐year survival of 77%, although this still reflects significant mortality . Use of the REVEAL risk score in the Lim et al .’s study had prognostic utility, although due to the relatively small numbers, the distinction between groups was simplified to ‘low risk’ and ‘high risk’ rather than the five sub‐categories published elsewhere .…”

mentioning

confidence: 99%

Pulmonary arterial hypertension: In Asia, as elsewhere, still a lethal disease despite modern treatment

Reynolds

2018

Respirology

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Survival of Idiopathic Pulmonary Arterial Hypertension Patients in the Modern Era in Australia and New Zealand

Cited by 35 publications

References 27 publications

Learning from registries in pulmonary arterial hypertension: pitfalls and recommendations

Learning from registries in pulmonary arterial hypertension: pitfalls and recommendations

Serum cardiac troponin elevation predicts mortality in patients with pulmonary hypertension: A meta‐analysis of eight cohort studies

Pulmonary arterial hypertension: In Asia, as elsewhere, still a lethal disease despite modern treatment

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