Validation of two predictive models for survival in pulmonary arterial hypertension

Olivier Sitbon, Raymond L. Benza, David B. Badesch, Robyn J. Barst, C. Gregory Elliott, Virginie Gressin, Jean Christophe Lemarié, Dave P. Miller, Erwan Muros Le Rouzic, Gérald Simonneau, Adaani E. Frost, Harrison W. Farber, Marc Humbert, Michael D. McGoon

Research output: Contribution to journalArticlepeer-review

75 Scopus citations


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 0.64-0.80, and 0.73, 95% CI 0.70-0.77, 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.

Original languageEnglish (US)
Pages (from-to)152-164
Number of pages13
JournalEuropean Respiratory Journal
Issue number1
StatePublished - Jul 1 2015

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine


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