Prognostic implications of serial risk score assessments in patients with pulmonary arterial hypertension: A Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL) analysis

Benza, Raymond L.; Miller, Dave P.; Foreman, Aimee J.; Frost, Adaani E.; Badesch, David B.; Benton, Wade W.; McGoon, Michael D.

doi:10.1016/j.healun.2014.09.016

Cited by 123 publications

(145 citation statements)

References 11 publications

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“…Although limited to a subset of patients (n=606) with SvO 2 measured at first re-evaluation, the presence of SvO 2 >65% predicted survival independently of WHO/NYHA functional class, 6MWD, RAP and cardiac index, again extending the results of NICKEL et al [15]. The REVEAL (Registry to Evaluate Early and Long-term PAH Disease Management) score is another validated risk assessment tool that can be applied serially during follow-up in PAH patients to predict the risk of death during the following 12 months; however, this calculated score uses 13 different variables, some of which are nonmodifiable, such as age, sex and aetiology of PAH [16][17][18][19].…”

Section: Discussionmentioning

confidence: 81%

Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension

et al. 2017

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@ERSpublicationsSimplified risk assessment using the number of low-risk criteria predicts prognosis at baseline and follow-up in PAH http://ow.ly/KMsj30cPNbmCite this article as: Boucly A, Weatherald J, Savale L, et al. Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension. Eur Respir J 2017; 50: 1700889 [https://doi.org/ 10.1183/13993003.00889-2017.ABSTRACT Current European guidelines recommend periodic risk assessment for patients with pulmonary arterial hypertension (PAH). The aim of our study was to determine the association between the number of low-risk criteria achieved within 1 year of diagnosis and long-term prognosis.Incident patients with idiopathic, heritable and drug-induced PAH between 2006 and 2016 were analysed. The number of low-risk criteria present at diagnosis and at first re-evaluation were assessed: World Health Organization (WHO)/New York Heart Association (NYHA) functional class I or II, 6-min walking distance (6MWD) >440 m, right atrial pressure <8 mmHg and cardiac index ⩾2.5 L·min. 1017 patients were included (mean age 57 years, 59% female, 75% idiopathic PAH). After a median follow-up of 34 months, 238 (23%) patients had died. Each of the four low-risk criteria independently predicted transplant-free survival at first re-evaluation. The number of low-risk criteria present at diagnosis ( p<0.001) and at first re-evaluation ( p<0.001) discriminated the risk of death or lung transplantation. In addition, in a subgroup of 603 patients with brain natriuretic peptide (BNP) or Nterminal pro-brain natriuretic peptide (NT-proBNP) measurements, the number of three noninvasive criteria (WHO/NYHA functional class, 6MWD and BNP/NT-proBNP) present at first re-evaluation discriminated prognostic groups ( p<0.001).A simplified risk assessment tool that quantifies the number of low-risk criteria present accurately predicted transplant-free survival in PAH.

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Section: Discussionmentioning

confidence: 81%

Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension

et al. 2017

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“…It is important to note, however, that similarly high c-indexes have been achieved with single parameters in other studies [34] and there is considerable debate about whether use of multi-factor models provides sufficient benefit over individual factors to warrant the added complexity [24]. The potential importance of evaluating risk of death in PAH based on multifactorial assessment compared with a single parameter has been shown in several analyses using the REVEAL risk score [24,35]. These models may offer a prognosis better fitted to a patient's individual level of risk that might not be captured from a single clinical parameter.…”

Section: Discussionmentioning

confidence: 97%

“…Data from the REVEAL and FPHN registries, and the two equations derived from them, have been useful to compare patients' characteristics, predict outcomes in two different populations, and test the validity of prognostic indicators such as FC, 6MWD and cardiac index. However, the use of the REVEAL risk score calculator and the FPHN ItinérAIR-HTAP equation in routine clinical practice requires further evaluation in patients with PAH, with prospective experimental designs rather than observational [35].…”

Section: Discussionmentioning

confidence: 99%

Validation of two predictive models for survival in pulmonary arterial hypertension

et al. 2015

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The French Pulmonary Hypertension Network (FPHN) registry and the Registry to Evaluate Early And Long-term Pulmonary Arterial Hypertension Disease Management (REVEAL) have developed predictive models for survival in pulmonary arterial hypertension (PAH). In this collaboration, we assess the external validity (or generalisability) of the FPHN ItinérAIR-HTAP predictive equation and the REVEAL risk score calculator.Validation cohorts approximated the eligibility criteria defined for each model. The REVEAL cohort comprised 292 treatment-naïve, adult patients diagnosed <1 year prior to enrolment with idiopathic, familial or anorexigen-induced PAH. The FPHN cohort comprised 1737 patients with group 1 PAH.Application of FPHN parameters to REVEAL and REVEAL risk scores to FPHN demonstrated estimated hazard ratios that were consistent between studies and had high probabilities of concordance (hazard ratios of 0.72, 95% CI 0. and 0.73, respectively).The REVEAL risk score calculator and FPHN ItinérAIR-HTAP predictive equation showed good discrimination and calibration for prediction of survival in the FPHN and REVEAL cohorts, respectively, suggesting prognostic generalisability in geographically different PAH populations. Once prospectively validated, these may become valuable tools in clinical practice. @ERSpublications REVEAL risk score and ItinérAIR-HTAP equation show geographic validity (generalisability) in distinct PAH populations http://ow.ly/INyx8

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“…To be statistically sound, the risk assessment tool must have good discrimination and calibration; be derived from sizable well-defined cohorts; be composed of risk parameters (and corresponding cut points) that are evidence-based rather than expert opinion-derived; incorporate "weighting" of the various parameters; and be validated internally and externally. 69 The National Institutes of Health (NIH) registry equation, 6 reappraised in later work from the Pulmonary Hypertension Connection (PHC) registry, 70 the Scottish Composite Score, 28 as well as those from the larger French Pulmonary Arterial Hypertension Network (FPHN ItinérAIR-HTAP) registry 71,72 and REVEAL (USA) 8,20,70,73 have collectively informed current treatment guidelines by facilitating the creation of the current European Society of Cardiology (ESC)/ European Respiratory Society (ERS) risk table as well as the recommendations regarding periodic assessment of patient FC, BNP level, 6MWD, and right heart catheterization to stratify risk. 4 Data from these registries were used to derive equations and tools that predict survival for patients with PAH.…”

Section: Risk Equations Tables and Toolsmentioning

confidence: 99%

“…8,20 The REVEAL risk score calculator (Figure 7) was designed to help stratify patients' mortality risk and allow clinicians to make informed treatment decisions based on the unique risk profile of individual patients. The RE-VEAL predictive algorithm and simplified risk score calculator were internally validated in the newly diagnosed patients in REVEAL, 20 demonstrated utility in serial risk assessments, 73 and were externally validated in FPHN ItinérAIR-HTAP, 71 UK, 74 Spanish (REHAP), 75 and Mayo Clinic 76 registries, as well as several institutional databases enriched in specific PAH subgroups. 77 Furthermore, the REVEAL risk score calculator was inherently designed to accommodate periodic updates based on emerging data from PAH registries and clinical trials.…”

Section: Risk Equations Tables and Toolsmentioning

confidence: 99%

Risk Assessment in Pulmonary Arterial Hypertension Patients: The Long and Short of it

Benza

Lohmueller

Kraisangka

et al. 2018

Advances in Pulmonary Hypertension

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Pulmonary arterial hypertension (PAH) is a chronic and rapidly progressive disease that is characterized by extensive narrowing of the pulmonary vasculature, leading to increases in pulmonary vascular resistance, subsequent right ventricular dysfunction, and eventual death. There are currently multiple approved drugs—developed as single or combination therapies in the last few years—that have improved outcome and functionality in PAH. However, despite improvement in short-term survival with these new effective therapies, PAH remains an incurable disease with a median survival of 7 years (Figure 1).1 This chronic disease state may be characterized by morbid events such as hospitalizations that herald rapid disease progression and account for a significant disease burden (Figure 2).23 Physician ability to predict PAH disease progression is critical for determining optimal care of patients. Accurate risk assessment allows clinicians to determine the patient's prognosis, identify treatment goals, and monitor disease progression and the patient's response to treatment. Risk assessment for PAH patients should include a range of clinical, hemodynamic, and exercise parameters, performed in a serial fashion over the treatment course. Patient risk stratification can also help physicians better allocate treatment resources in settings where they are scarce. If widely adopted, risk prediction can enhance the consistency of treatment approaches across PAH practitioners and improve the timeliness of referral for lung transplantation. Hence, along with advancing PAH treatment options, comprehensive risk prediction is essential to make individualized treatment decisions in the current treatment era. Several tools are currently available for assessing risk in PAH (Figure 3). These include the 2015 European Society of Cardiology/European Respiratory Society pulmonary hypertension guidelines' risk variables,4 the French registry equation,5 the National Institutes of Health risk equation,6 or a risk score such as the one derived from the Registry to Evaluate Early And Long-term PAH Disease Management.1 These registries and evaluations of clinical trial sets have provided important insights into the importance of both modifiable (eg, 6-minute walk distance, functional class, brain natriuretic peptide, and nonmodifiable (eg, age, gender, PAH etiology) risk factors that predict survival. The following review explores commonly cited risk factors, both modifiable and nonmodifiable, and their implications for patient outcomes.

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Prognostic implications of serial risk score assessments in patients with pulmonary arterial hypertension: A Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Management (REVEAL) analysis

Abstract: Changes in REVEAL risk scores occur in most patients with pulmonary arterial hypertension over a 12-month period and are predictive of survival. Thus, serial risk score assessments can identify changes in disease trajectory that may warrant treatment modifications.

Cited by 123 publications

References 11 publications

Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension

Risk assessment, prognosis and guideline implementation in pulmonary arterial hypertension

Validation of two predictive models for survival in pulmonary arterial hypertension

Risk Assessment in Pulmonary Arterial Hypertension Patients: The Long and Short of it

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