title: Validation of two predictive models for survival in pulmonary arterial hypertension.
abstract: 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.
authors.affiliation: Université Paris-Sud, CHU de Bicêtre, INSERM U999, Le Kremlin Bicêtre, France Both authors contributed equally olivier.sitbon@bct.aphp.fr.
authors.forename: Olivier
authors.initials: O
authors.lastname: Sitbon
authors.affiliation: The Cardiovascular Institute, West Penn Allegheny Health System, Pittsburgh, PA, USA Both authors contributed equally.
authors.forename: Raymond L
authors.initials: RL
authors.lastname: Benza
authors.affiliation: Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado Health Sciences Center, Denver, CO, USA.
authors.forename: David B
authors.initials: DB
authors.lastname: Badesch
authors.affiliation: Columbia University College of Physicians and Surgeons, New York, NY, USA.
authors.forename: Robyn J
authors.initials: RJ
authors.lastname: Barst
authors.affiliation: Intermountain Medical Center and the University of Utah, Salt Lake City, UT, USA.
authors.forename: C Gregory
authors.initials: CG
authors.lastname: Elliott
authors.affiliation: Actelion Pharmaceuticals France, Paris, France.
authors.forename: Virginie
authors.initials: V
authors.lastname: Gressin
authors.affiliation: Effi-stat, Paris, France.
authors.forename: Jean-Christophe
authors.initials: JC
authors.lastname: Lemarié
authors.affiliation: ICON Clinical Research, San Francisco, CA, USA.
authors.forename: Dave P
authors.initials: DP
authors.lastname: Miller
authors.affiliation: Actelion Pharmaceuticals, Allschwil, Switzerland.
authors.forename: Erwan
authors.initials: E
authors.lastname: Muros-Le Rouzic
authors.affiliation: Université Paris-Sud, CHU de Bicêtre, INSERM U999, Le Kremlin Bicêtre, France.
authors.forename: Gérald
authors.initials: G
authors.lastname: Simonneau
authors.affiliation: Baylor College of Medicine, Houston, TX, USA.
authors.forename: Adaani E
authors.initials: AE
authors.lastname: Frost
authors.affiliation: Boston University School of Medicine, Boston, MA, USA.
authors.forename: Harrison W
authors.initials: HW
authors.lastname: Farber
authors.affiliation: Université Paris-Sud, CHU de Bicêtre, INSERM U999, Le Kremlin Bicêtre, France Both authors contributed equally.
authors.forename: Marc
authors.identifier: http://orcid.org/0000-0003-0703-2892
authors.initials: M
authors.lastname: Humbert
authors.affiliation: Mayo Clinic, Rochester, MN, USA Both authors contributed equally.
authors.forename: Michael D
authors.initials: MD
authors.lastname: McGoon
mesh_terms: D000328:Adult; D000368:Aged; D000465:Algorithms; D002138:Calibration; D015331:Cohort Studies; D065627:Familial Primary Pulmonary Hypertension / Q000175:diagnosis* / Q000401:mortality*; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000401:mortality*; D015994:Incidence; D008297:Male; D008875:Middle Aged; D008962:Models, Theoretical; D011237:Predictive Value of Tests; D011336:Probability; D016016:Proportional Hazards Models; D012042:Registries; D012306:Risk; D015996:Survival Rate