Retrospective Validation of the REVEAL 2.0 Risk Score With the Australian and New Zealand Pulmonary Hypertension Registry Cohort

Anderson, James; Lau, Edmund; Lavender, Melanie; Benza, Raymond L.; Celermajer, David S.; Collins, Nicholas; Corrigan, Carolyn; Dwyer, Nathan; Feenstra, John; Horrigan, Mark; Keating, D.; Kermeen, F.; Kotlyar, Eugene; McWilliams, Tanya; Rhodes, Bronwen; Steele, Peter; Thakkar, Vivek; Williams, Trevor; Whitford, Helen; Whyte, K.; Weintraub, Robert; Wrobel, Jérémy; Keogh, Anne; Strange, Geoff

doi:10.1016/j.chest.2019.08.2203

Cited by 27 publications

(27 citation statements)

References 24 publications

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“…12 Additions to and adjustment of certain variables led to the development of REVEAL 2.0, 13 which was also validated. 14 The phase III GRIPHON study, 15 the largest event-driven outcome trial to date in PAH, allows evaluation of the ability of these risk assessment approaches to predict long-term morbidity/mortality outcomes in the context of a randomized, controlled trial and in a predominantly prevalent population. GRIPHON evaluated the efficacy and safety of selexipag, an oral, selective prostacyclin receptor (IP) agonist, in 1,156 patients.…”

Section: Discussionmentioning

confidence: 99%

“…The prognostic value of REVEAL 2.0 has been validated in a registry of predominantly prevalent patients. 14 The association between risk and outcome has also been investigated using clinical trial data. A recent post-hoc analysis of data from 340 patients in the open-label extension trial PAH: a long-term Effect of selexipag on the primary composite end-point of morbidity/mortality by REVEAL 2.0 risk category.…”

Section: Discussionmentioning

confidence: 99%

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Risk assessment in pulmonary arterial hypertension: Insights from the GRIPHON study

Sitbon

Chin

Channick

et al. 2020

The Journal of Heart and Lung Transplantation

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BACKGROUND: Approaches to risk assessment in pulmonary arterial hypertension (PAH) include the noninvasive French risk assessment approach (number of low-risk criteria based on the European Society of Cardiology and European Respiratory Society guidelines) and Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL) 2.0 risk calculator. The prognostic and predictive value of these methods for morbidity/mortality was evaluated in the predominantly prevalent population of GRIPHON, the largest randomized controlled trial in PAH. METHODS: GRIPHON randomized 1,156 patients with PAH to selexipag or placebo. Post-hoc analyses were performed on the primary composite end-point of morbidity/mortality by the number of low-risk criteria (World Health Organization functional class I-II; 6-minute walk distance >440 m; N-terminal pro-brain natriuretic peptide <300 ng/liter) and REVEAL 2.0 risk category. Hazard ratios and 95% confidence intervals were calculated using Cox proportional hazard models. RESULTS: Both the number of low-risk criteria and the REVEAL 2.0 risk category were prognostic for morbidity/mortality at baseline and any time-point during the study. Patients with 3 low-risk criteria at baseline had a 94% reduced risk of morbidity/mortality compared to patients with 0 low-risk criteria and were all categorized as low-risk by REVEAL 2.0. The treatment effect of selexipag on morbidity/ mortality was consistent irrespective of the number of low-risk criteria or the REVEAL 2.0 risk category at any time-point during the study. Selexipag-treated patients were more likely to increase their number of low-risk criteria from baseline to week 26 than placebo-treated patients (odds ratio 1.69, p = 0.0002); similar results were observed for REVEAL 2.0 risk score.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Risk assessment in pulmonary arterial hypertension: Insights from the GRIPHON study

Sitbon

Chin

Channick

et al. 2020

The Journal of Heart and Lung Transplantation

View full text Add to dashboard Cite

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“…In contrast to the ESC/ERS guidelines, REVEAL assigns an overall score based on multiple demographic, haemodynamic and serum parameters in order to establish risk of 1-year mortality, and has also been validated in multiple studies [9,[38][39][40][41]. An update, REVEAL 2.0, has recently been published [9] which, in an independent cohort, demonstrated 1-year mortality estimates of 2.6%, 8.6% and 25.4% for low-, intermediate-and high-risk groups respectively, with 5-year estimates of 16.1%, 41.5% and 88.0%, respectively [41]. Additionally, a study using CMR of RV end systolic volume index adjusted for age and sex has been shown to further improve risk stratification for 1-year mortality when used with either the REVEAL 2.0 score or a modified French Pulmonary Hypertension Risk score [13].…”

Section: Bnp/nt-probnp and Comparison With Pulmonary Haemodynamics Ementioning

confidence: 99%

BNP/NT-proBNP in pulmonary arterial hypertension: time for point-of-care testing?

et al. 2020

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Despite the advent of new therapies and improved outcomes in patients with pulmonary arterial hypertension (PAH), it remains a life-shortening disease and the time to diagnosis remains unchanged. Strategies to improve outcomes are therefore currently focused on earlier diagnosis and a treatment approach aimed at moving patients with PAH into a category of low-risk of 1-year mortality. B-type natriuretic peptide (BNP; or brain natriuretic peptide) and N-terminal prohormone of BNP (NT-proBNP) are released from cardiac myocytes in response to mechanical load and wall stress. Elevated levels of BNP and NT-proBNP are incorporated into several PAH risk stratification tools and screening algorithms to aid diagnosis of systemic sclerosis. We have undertaken a systematic review of the literature with respect to the use of BNP and NT-proBNP in PAH and the use of these biomarkers in the diagnosis and risk stratification of PAH, their relation to pulmonary haemodynamics and the potential for point-of-care testing to improve diagnosis and prognosis.

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“…Furthermore, in our study, risk stratification approaches were applied to treatment-naïve patients rather than a mixture of incident and prevalent patients as in the REVEAL study. These factors, and particularly the absence of patients with PAH-CHD (the presence of which scores -2 points in REVEAL 2.0) may also explain why a relatively small number of our patients were identified as being at low-risk by REVEAL 2.0 when compared to the original study and external validation studies (28,39).…”

Section: ≥340mmentioning

confidence: 92%

Maximal Exercise Testing Using the Incremental Shuttle Walking Test Can Be Used to Risk-Stratify Patients with Pulmonary Arterial Hypertension

et al. 2021

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Rationale: Exercise capacity predicts mortality in pulmonary arterial hypertension but limited data exist on the routine use of maximal exercise testing. Objectives: This study evaluates a simple to perform maximal test, the incremental shuttle walking test, and its utility in risk stratification in pulmonary arterial hypertension (PAH). Methods: Consecutive patients with pulmonary hypertension were identified from the ASPIRE registry (2001-2018). Thresholds for levels of risk were identified at baseline, tested at followup and incorporation into current risk stratification approaches assessed. Results: Of 4524 treatment-naïve patients with pulmonary hypertension who underwent maximal exercise testing 1,847 patients had PAH. A step-wise reduction in one-year-mortality was seen between levels 1 (≤30m; 32% mortality) and 7 (340-420m; 1% mortality) with no mortality for levels 8-12 (≥430m) in idiopathic and connective tissue disease related PAH. Thresholds derived at baseline of ≤180m (>10%; high-risk), 190-330m (5-10%; intermediaterisk) and ≥340m (<5%; low-risk of one-year mortality) were applied at follow-up and also accurately identified levels of risk. Thresholds were incorporated into the REVEAL 2.0 risk score calculator and French low-risk approach to risk stratification and distinct categories of risk remained. Conclusion: We have demonstrated that maximal exercise testing in PAH stratifies mortalityrisk at baseline and follow-up. This study highlights the potential value of the incremental shuttle walking test as an alternative to the 6-minute-walk-test, combining some of the advantages of maximal exercise testing whilst maintaining the simplicity of a simple to perform field test.

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Retrospective Validation of the REVEAL 2.0 Risk Score With the Australian and New Zealand Pulmonary Hypertension Registry Cohort

Cited by 27 publications

References 24 publications

Risk assessment in pulmonary arterial hypertension: Insights from the GRIPHON study

Risk assessment in pulmonary arterial hypertension: Insights from the GRIPHON study

BNP/NT-proBNP in pulmonary arterial hypertension: time for point-of-care testing?

Maximal Exercise Testing Using the Incremental Shuttle Walking Test Can Be Used to Risk-Stratify Patients with Pulmonary Arterial Hypertension

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