Performance of the ESC/ERS 4-strata risk stratification model with missing variables

The 2022 European Society of Cardiology (ESC)/ European Respiratory Society (ERS) pulmonary hypertension (PH) guidelines recommend risk stratification to guide treatment decisions in patients with pulmonary arterial hypertension (PAH) [1, 2]. Risk, referring to the likelihood of death within 12 months, is estimated based on WHO functional class (FC), 6-minute walk distance (6MWD), and serum levels of brain natriuretic peptide (BNP) or N-terminal pro-BNP (NT-proBNP), using prespecified thresholds. A 3-strata model (categorizing risk as low, intermediate, or high) is recommended at time of diagnosis, whereas a 4-strata model (categorizing risk as low, intermediate-low, intermediate-high, or high) is recommended during the course of the disease. The 4-strata model was developed from the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hypertension (COMPERA) and cross-validated by the French PH registry [3, 4]. Both studies included patients when all three variables, i.e. , WHO-FC, 6MWD, and BNP/NT-proBNP were available at baseline. No formal assessment of the predictive value of the 4-strata model has been performed when one of these components were missing. Footnotes This manuscript has recently been accepted for publication in the European Respiratory Journal . It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article. Conflict of interest: Christine Pausch has no disclosures. Conflict of interest: David Pittrow has received fees for consultations from Actelion, Amgen, Aspen, Bayer, Biogen, Boehringer Ingelheim, Daiichi Sankyo, MSD, Novartis, Sanofi-Genzyme, Takeda, Viatris and Zambon. Conflict of interest: Marius M. Hoeper has received fees for lectures and/or consultations from Acceleron, Actelion, AOP Health, Bayer, Ferrer, GSK, Janssen, MSD, and Pfizer. Conflict of interest: Doerte Huscher has no disclosures.