RT Journal Article SR Electronic T1 Predicting mortality during long-term follow-up in pulmonary arterial hypertension JF ERJ Open Research JO erjor FD European Respiratory Society SP 00837-2020 DO 10.1183/23120541.00837-2020 A1 David Kylhammar A1 Clara Hjalmarsson A1 Roger Hesselstrand A1 Kjell Jansson A1 Mohammad Kavianipour A1 Barbro Kjellström A1 Magnus Nisell A1 Stefan Söderberg A1 Göran Rådegran A1 , YR 2021 UL http://openres.ersjournals.com/content/early/2021/01/14/23120541.00837-2020.abstract AB The European Society of Cardiology (ESC) and European Respiratory Society (ERS) guideline recommendation of comprehensive risk assessments, which classify patients with pulmonary arterial hypertension (PAH) as having low, intermediate or high mortality risk, has not been evaluated during long-term follow-up in a “real-life” clinical setting. We therefore aimed to investigate the utility of risk assessment in a clinical setting for up to 5 years post diagnosis.Three hundred and eighty-six patients with PAH from the Swedish PAH Registry were included. Risk group (low/intermediate/high) and proportion of low risk variables were investigated at 3-, 4- and 5-year follow-ups after time of diagnosis. In an exploratory analysis, survival rates of patients with low- or high intermediate risk scores were compared.A low risk profile was in multivariate Cox proportional hazards regressions found to be a strong, independent predictor of longer transplant-free survival (p<0.001) at the 3-, 4- and 5-year follow-ups. Also, for the 3-, 4- and 5-year follow-ups, survival rates significantly differed (p<0.001) between the three risk groups. Patients with a greater proportion of low risk variables had better (p<0.001) survival rates. Patients with a high intermediate risk score had worse survival rates (p<0.001) than those with a low intermediate risk score. Results were similar when excluding patients with ≥3 risk factors for heart failure with preserved ejection fraction, atrial fibrillation and/or age >75 years at diagnosis.Our findings suggest that the ESC/ERS guideline strategy for comprehensive risk assessments in PAH is valid also during long-term follow-up in a “real-life” clinical setting.FootnotesThis manuscript has recently been accepted for publication in the ERJ Open Research. 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 ERJOR online. Please open or download the PDF to view this article.Conflict of interest: D. Kylhammar reports grants from The Swedish Association for Pulmonary Hypertension in cooperation with Bayer Health Care, and lecture fees from Actelion Pharmaceuticals Sweden AB and Glaxo-SmithKline, outside the submitted work.Conflict of interest: C. Hjalmarsson reports grants and personal fees for research advisory boards from Actelion Pharmaceuticals Sweden AB, and grants from MSD, outside the submitted work.Conflict of interest: Dr. Hesselstrand has nothing to disclose.Conflict of interest: Dr. Jansson has nothing to disclose.Conflict of interest: Dr. Kavianipour has nothing to disclose.Conflict of interest: Dr. Kjellström has nothing to disclose.Conflict of interest: M. Nisell reports lecture and consultation fees from Actelion Pharmaceuticals Sweden AB, Pfizer, Bayer HealthCare, NordicInfu Care and GlaxoSmith-Kline outside the submitted work.Conflict of interest: S. Söderberg reports grants and lecture fees from Actelion Pharmaceuticals Sweden AB, and grants from Pfizer, outside the submitted work.Conflict of interest: G. Rådegran reports grants, lecture fees and research advisory boards from Actelion Pharmaceuticals Sweden AB and Glaxo-SmithKline; lecture fees and research advisory boards from Bayer Health Care; lecture fees from Nordic InfuCare and Sandoz/Novartis; and research advisory boards for Acceleron, Arena, Eli-Lilly and Sanofi-Aventis, all outside the submitted work.