REVEAL risk scores applied to riociguat-treated patients in PATENT-2: Impact of changes in risk score on survival

Benza, Raymond L.; Farber, Harrison W.; Frost, Adaani E.; Ghofrani, Hossein Ardeschir; Gómez-Sánchez, Miguel Ángel; Langleben, David; Rosenkranz, Stephan; Busse, Dennis; Meier, Christian; Nikkho, Sylvia; Hoeper, Marius M.

doi:10.1016/j.healun.2017.11.006

Cited by 31 publications

(61 citation statements)

References 21 publications

(34 reference statements)

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“…17,18 Although the post hoc nature of this study could be considered a potential limitation, it is important to note it is unusual (outside of the clinical trial setting) for patients to undergo the extensive battery of tests required to predict risk at every clinic visit, and therefore the RRS was designed to predict risk based on the most recent data available. 19 The RRS has been validated by post hoc calculation in the French Pulmonary Hypertension Network registry cohort, 26 and also in the PATENT 27 and SERAPHIN 28 clinical studies in patients with PAH. Additional limitations include the lack of validation of the RRS in CTEPH, and the high proportion of low-risk patients at baseline.…”

Section: Discussionmentioning

confidence: 99%

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

Benza

Farber

Frost

et al. 2018

The Journal of Heart and Lung Transplantation

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Section: Discussionmentioning

confidence: 99%

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

Benza

Farber

Frost

et al. 2018

The Journal of Heart and Lung Transplantation

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“…[4][5][6] In post hoc analyses, interventions in clinical trials have been shown to correlate with improvements in the REVEAL risk score. 17 Validation of risk assessment strategies as a surrogate end point should be explored in future clinical trials. 6…”

Section: Discussionmentioning

confidence: 99%

“…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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“…4 Preliminary work suggests it could be useful as an endpoint in clinical trials. 7 An important lesson and recurring theme from multiple prognostication studies is that many patients are not treated aggressively despite intermediate or high-risk features, and despite the evolving data to indicate that more aggressive up-front or early combination therapy and more aggressive use of parenteral prostanoids in higher-risk patients is associated with improved outcomes. In this light, a few high-level recommendations should be considered when deciding on initial treatment options.…”

Section: Step 5: Create An Initial Treatment Strategymentioning

confidence: 99%

Treatment Considerations for Pulmonary Arterial Hypertension and Assessment of Treatment Response

Frantz

2018

Advances in Pulmonary Hypertension

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The process of considering initial treatment options for a patient with pulmonary arterial hypertension (PAH), implementing that treatment, and reassessing response can be conceived as a 7-step process. A patient-centered approach, conducted in the context of a pulmonary hypertension care team, implements these steps in a thoughtful and well-organized fashion.Step 1: Perform a thorough diagnostic evaluationStep 2: Consider patient PAH classification and comorbiditiesStep 3: Assess risk profileStep 4: Develop a nuanced understanding of patient goals and preferencesStep 5: Create an initial treatment strategyStep 6: Implement a proactive longitudinal follow-up care planStep 7: Adjust treatment approach based on clear metrics of effectiveness and tolerability The burgeoning array of treatment options for PAH provides the opportunity to tailor therapy to the needs of individual patients, but also demands that the clinician develop a cogent strategy for initiating therapy, monitoring therapeutic response, and addressing side effects of therapy. In this review, we seek to provide a practical framework for approaching this challenge.

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REVEAL risk scores applied to riociguat-treated patients in PATENT-2: Impact of changes in risk score on survival

Abstract: RRS at baseline and Week 12, and change in RRS, were significant predictors of both survival and clinical worsening-free survival. These data support the long-term predictive value of the RRS in a controlled study population.

Cited by 31 publications

References 21 publications

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

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

Predicting Survival in Patients With Pulmonary Arterial Hypertension

Treatment Considerations for Pulmonary Arterial Hypertension and Assessment of Treatment Response

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