Chest
Volume 156, Issue 2, August 2019, Pages 323-337
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Original Research: Pulmonary and Cardiovascular
Predicting Survival in Patients With Pulmonary Arterial Hypertension: The REVEAL Risk Score Calculator 2.0 and Comparison With ESC/ERS-Based Risk Assessment Strategies

Preliminary data were presented at the International Society for Heart and Lung Transplantation 37th Annual Meeting, April 5-8, 2017, San Diego, CA; The American Thoracic Society International Conference, May 19-24, 2017, Washington DC; and at The American Thoracic Society International Conference, May 18-23, 2018, San Diego, CA.
https://doi.org/10.1016/j.chest.2019.02.004Get rights and content
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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 = 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 = 0.73) than COMPERA (c-statistic = 0.62) or FPHR (c-statistic = 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.

Trial Registry

ClinicalTrials.gov; No. NCT00370214; URL: www.clinicaltrials.gov.

Key Words

ESC/ERS-derived risk assessment
pulmonary arterial hypertension
registry
REVEAL
risk score calculator

Abbreviations

6MWD
6-min walk distance
BNP
brain natriuretic peptide
COMPERA
Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension
Dlco
diffusing capacity of the lungs for carbon monoxide
eGFR
estimated glomerular filtration rate
ERS
European Respiratory Society
ESC
European Society of Cardiology
FC
functional class
FPHR
French Pulmonary Hypertension Registry
PAH
pulmonary arterial hypertension
PVR
pulmonary vascular resistance
RAP
right atrial pressure
REVEAL
Registry to Evaluate Early and Long-Term PAH Disease Management
SPAHR
Swedish Pulmonary Arterial Hypertension Register
TIMI
thrombolysis in myocardial infarction
WHO
World Health Organization

Cited by (0)

FUNDING/SUPPORT: Actelion Pharmaceuticals US, Inc. (A Janssen Pharmaceutical Company of Johnson and Johnson) is the sponsor of the Registry to Evaluate Early and Long-Term PAH Disease Management Registry and provided funding and support for the analysis presented.