REVEAL risk score in patients with chronic thromboembolic pulmonary hypertension receiving riociguat

Benza, Raymond L.; Farber, Harrison W.; Frost, Adaani E.; Grünig, Ekkehard; Hoeper, Marius M.; Busse, Dennis; Meier, Christian; Nikkho, Sylvia; Ghofrani, Hossein Ardeschir

doi:10.1016/j.healun.2018.02.015

Cited by 33 publications

(38 citation statements)

References 29 publications

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“…However, each of the three abbreviated tools in the current post hoc analysis had the ability to discriminate prognosis for survival and clinical worsening-free survival among patients with inoperable or persistent/recurrent CTEPH following 16 weeks of treatment with riociguat, although further analyses would need to be performed to statistically compare the discriminatory ability of the tools. These observations are in line with similar results found when the RRS was applied to the CHEST study database, in which riociguat significantly improved RRS and risk stratum versus placebo from baseline to week 16 [16]. In addition, RRS at baseline and week 16, and change in RRS during CHEST-1, were significantly associated with survival and clinical worsening-free survival over 2 years in CHEST-2.…”

Section: Discussionsupporting

confidence: 88%

“…There is currently no recommendation regarding how to assess risk in patients with inoperable CTEPH; however, in addition to its common and well-validated use in assessing risk in patients with PAH, the REVEAL risk score (RRS) has shown utility as a prognostic tool in patients with CTEPH [16] and a recent study suggested that the Swedish/COMPERA method predicts mortality in non-operated patients with CTEPH [17].…”

Section: Introductionmentioning

confidence: 99%

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Risk assessment in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension

et al. 2019

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BackgroundCurrent pulmonary hypertension treatment guidelines recommend use of a risk stratification model encompassing a range of parameters, allowing patients to be categorised as low, intermediate or high risk. Three abbreviated versions of this risk stratification model were previously evaluated in patients with pulmonary arterial hypertension (PAH) in the French, Swedish and COMPERA registries. Our objective was to investigate the three abbreviated risk stratification methods for patients with mostly prevalent PAH and chronic thromboembolic pulmonary hypertension (CTEPH), in patients from the PATENT-1/2 and CHEST-1/2 studies of riociguat.MethodsRisk was assessed at baseline and at follow-up in PATENT-1 and CHEST-1. Survival and clinical worsening-free survival were assessed in patients in each risk group/strata.ResultsWith all three methods, riociguat improved risk group/strata in patients with PAH after 12 weeks. The French non-invasive and Swedish/COMPERA methods discriminated prognosis for survival and clinical worsening-free survival at both baseline and follow-up. Furthermore, patients achieving one or more low-risk criteria or a low-risk stratum at follow-up had a significantly reduced risk of death and clinical worsening compared with patients achieving no low-risk criteria or an intermediate-risk stratum. Similar results were obtained in patients with inoperable or persistent/recurrent CTEPH.ConclusionsThis analysis confirms and extends the results of the registry analyses, supporting the value of goal-oriented treatment in PAH. Further assessment of these methods in patients with CTEPH is warranted.

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Section: Discussionsupporting

confidence: 88%

Section: Introductionmentioning

confidence: 99%

Risk assessment in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension

et al. 2019

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“…In the first 12 weeks of CHEST-2, this improvement continued in patients who received riociguat in CHEST-1 and former placebo patients who started receiving riociguat in CHEST-2 also demonstrated improvement similar to riociguat patients in CHEST-1. 24 The RRS as well as change in the RRS was shown to be a significant predictor of survival and clinical worsening-free survival over 2-years in CHEST-2. 24 …”

Section: Riociguat For the Treatment Of Ctephmentioning

confidence: 87%

“… 24 The RRS as well as change in the RRS was shown to be a significant predictor of survival and clinical worsening-free survival over 2-years in CHEST-2. 24 …”

Section: Riociguat For the Treatment Of Ctephmentioning

confidence: 87%

<p>Riociguat in the Treatment of Chronic Thromboembolic Pulmonary Hypertension: An Evidence-Based Review of Its Place in Therapy</p>

Donaldson¹,

Ogunti²,

Kibreab³

et al. 2020

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“…7,14 The REVEAL risk calculator predicts survival in diverse PAH populations and provides useful serial survival assessments. 7,10,[13][14][15][16][17][18][19][20] More recently, investigators have derived three additional risk assessment strategies using incident patient populations from the Swedish Pulmonary Arterial Hypertension Register (SPAHR), 21 the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA), 22 and the French Pulmonary Hypertension Registry (FPHR). 23 These risk assessment strategies are made on the basis of thresholds defined by the European Society of Cardiology and European Respiratory Society (ESC/ ERS) 4 in four to eight variables, and are calculated by various methods.…”

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confidence: 99%

Predicting Survival in Patients With Pulmonary Arterial Hypertension

Benza

Gomberg‐Maitland

Elliott

et al. 2019

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BACKGROUND: Pulmonary arterial hypertension is a progressive, fatal disease. Published treatment guidelines recommend treatment escalation on the basis of regular patient assessment with the goal of achieving or maintaining low-risk status. Various strategies are available to determine risk status. This analysis describes an update of the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL) risk calculator (REVEAL 2.0) and compares it with recently published European Society of Cardiology/ Respiratory Society guideline-derived risk assessment strategies. METHODS: A subpopulation from the US-based registry REVEAL that survived $ 1 year postenrollment (baseline for this cohort) was analyzed. For REVEAL 2.0, point values and cutpoints were reassessed, and new variables were evaluated. The Kaplan-Meier method was used to estimate survival at 12 months postbaseline; discrimination was quantified using the c-statistic. Mortality estimates and discrimination were compared between REVEAL 2.0 and Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA) and French Pulmonary Hypertension Registry (FPHR) risk assessment strategies. For this comparison, a three-category REVEAL 2.0 score was computed in which patients were classified as low-, intermediate-, or high-risk. RESULTS: REVEAL 2.0 demonstrated similar discrimination as the original calculator in this subpopulation (c-statistic ¼ 0.76 vs 0.74), provided excellent separation of risk among the risk categories, and predicted clinical worsening as well as mortality in patients who were followed $ 1 year. The REVEAL 2.0 three-category score had greater discrimination (c-statistic ¼ 0.73) than COMPERA (c-statistic ¼ 0.62) or FPHR (c-statistic ¼ 0.64). Compared with REVEAL 2.0, COMPERA and FPHR both underestimated and overestimated risk. CONCLUSIONS: REVEAL 2.0 demonstrates greater risk discrimination than the COMPERA and FPHR risk assessment strategies in patients enrolled in REVEAL. After external validation, the REVEAL 2.0 calculator can assist clinicians and patients in making informed treatment decisions on the basis of individual risk profiles.

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REVEAL risk score in patients with chronic thromboembolic pulmonary hypertension receiving riociguat

Cited by 33 publications

References 29 publications

Risk assessment in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension

Risk assessment in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension

<p>Riociguat in the Treatment of Chronic Thromboembolic Pulmonary Hypertension: An Evidence-Based Review of Its Place in Therapy</p>

Predicting Survival in Patients With Pulmonary Arterial Hypertension

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