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Original Research: PULMONARY VASCULAR DISEASE |

The REVEAL Registry Risk Score Calculator in Patients Newly Diagnosed With Pulmonary Arterial HypertensionValidation of the REVEAL Registry Risk Calculator

Raymond L. Benza, MD; Mardi Gomberg-Maitland, MD, FCCP; Dave P. Miller, MS; Adaani Frost, MD, FCCP; Robert P. Frantz, MD; Aimee J. Foreman, MA; David B. Badesch, MD, FCCP; Michael D. McGoon, MD, FCCP
Author and Funding Information

From The Gerald McGinnis Cardiovascular Institute, Allegheny General Hospital (Dr Benza), Pittsburgh, PA; the Department of Medicine, Section of Cardiology, University of Chicago Medical Center (Dr Gomberg-Maitland), Chicago, IL; Statistical Analysis, ICON Late Phase & Outcomes Research (Mr Miller and Ms Foreman), San Francisco, CA; Department of Medicine, Section of Pulmonary, Critical Care, and Sleep Medicine, Baylor College of Medicine (Dr Frost), Houston, TX; Division of Cardiovascular Diseases, Mayo Clinic (Drs Frantz and McGoon), Rochester, MN; and Department of Medicine, Division of Pulmonary Sciences and Critical Care Medicine, and Cardiology, University of Colorado (Dr Badesch), Denver, CO.

Correspondence to: Raymond L. Benza, MD, The Gerald McGinnis Cardiovascular Institute, Allegheny General Hospital, 320 E North Ave, 16th Floor, South Tower, Pittsburgh, PA 15212; e-mail: RBENZA@wpahs.org


Funding/Support: Funding for the REVEAL Registry is provided by Actelion Pharmaceuticals US, Inc.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (http://www.chestpubs.org/site/misc/reprints.xhtml).


© 2012 American College of Chest Physicians


Chest. 2012;141(2):354-362. doi:10.1378/chest.11-0676
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Background:  In pulmonary arterial hypertension (PAH), survival predictions can be important for optimization of therapeutic strategies. The present study aimed to validate a quantitative algorithm for predicting survival derived from the Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL Registry) and develop a simplified calculator for everyday clinical use.

Methods:  Prospectively collected data from patients with newly diagnosed (< 3 months) World Health Organization group I pulmonary hypertension enrolled in the REVEAL Registry were used to validate a predictive algorithm for 1-year survival. Model calibration was evaluated by comparing algorithm-predicted survival with observed Kaplan-Meier estimates for the overall validation cohort and for five risk groups. Similarly, the risk discriminators for the simplified calculator were compared with those of the quantitative algorithm.

Results:  The validation cohort comprised 504 individuals with mean ± SD 6-min walk distance 308 ± 128 m, and 61.5% were functional class III. The proportion of patients surviving 1 year fell within the range predicted by the model (95.1%, 91.5%, 84.6%, 76.3%, and 58.2%, respectively) among patients in the low (predicted survival ≥ 95%), average (90% to < 95%), moderate (85% to < 90%), high (70% to < 85%), and very high (< 70%) risk strata. Predicted and observed 1-year survival were similar across risk stratum, and the c-index indicated good discrimination for both the full equation (0.726) and the simplified risk calculator (0.724).

Conclusions:  The REVEAL Registry predictive algorithm and simplified risk score calculator are well calibrated and demonstrate good discriminatory ability in patients with newly or previously diagnosed PAH.

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

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