PT  - JOURNAL ARTICLE
AU  - Michael McGoon
AU  - Raymond Benza
AU  - Adaani Frost
AU  - David Badesch
AU  - C. Gregory Elliott
AU  - Olivier Sitbon
AU  - Gerald Simonneau
AU  - Dave Miller
AU  - Erwan Muros Le Rouzic
AU  - Marc Humbert
TI  - External validation of the French predictive model to estimate PAH survival: A REVEAL analysis
DP  - 2012 Sep 01
TA  - European Respiratory Journal
PG  - 368
VI  - 40
IP  - Suppl 56
4099  - http://erj.ersjournals.com/content/40/Suppl_56/368.short
4100  - http://erj.ersjournals.com/content/40/Suppl_56/368.full
SO  - Eur Respir J2012 Sep 01; 40
AB  - Background: The French Pulmonary Hypertension Network (FPHN) and Registry to EValuate Early And Long-term Pulmonary Arterial Hypertension (PAH) Disease Management (REVEAL) recently developed models to predict survival in PAH patients (pts).Aims and Objectives: The FPHN equation was tested in a REVEAL cohort to assess its generalizability in a different PAH population.Methods: The REVEAL validation cohort had 436 recently diagnosed (<1 year before enrollment), treatment-naïve, ≥18-year-old patients (pts) with idiopathic, familial, or anorexigen-induced PAH, divided into subgroups with non-missing (n=292) and missing (n=144) data for all FPHN equation parameters.Results: The FPHN and REVEAL cohorts had similar characteristics. FPHN follow-up (f/u) was ≥3 years; REVEAL validation cohort and subgroup with non-missing data had a mean f/u from diagnosis of 29.2 and 30.1 months, respectively. FPHN model parameters applied to REVEAL showed a good correlation of estimated hazard ratios between the two studies and robust C index of 0.72 (95% CI, 0.64–0.80). Risk stratification based on the FPHN equation showed good discrimination in REVEAL between high and low-risk pts. Survival in REVEAL correlated with FPHN equation predictions and was slightly better than predicted (Figure).Conclusion: The FPHN equation accurately stratified a matched US population according to risk, suggesting its prognostic generalizability in PAH pts.