{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,9]],"date-time":"2026-09-09T20:28:10Z","timestamp":1788985690798,"version":"build-2803163510"},"reference-count":41,"publisher":"MDPI AG","issue":"21","license":[{"start":{"date-parts":[[2021,10,31]],"date-time":"2021-10-31T00:00:00Z","timestamp":1635638400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Background: Right ventricular dysfunction is a major determinant of outcome in pulmonary arterial hypertension (PAH). We aimed to identify echocardiographic right heart parameters associated with adverse outcome and to develop a non-invasive, echocardiography-based risk score for PAH patients. Methods and Results: In 254 PAH patients we analyzed functional status, laboratory results, and echocardiographic parameters. We included these parameters to estimate all-cause death or lung transplantation using Cox regression models. The analyses included a conventional model using guideline-recommended variables and an extended echocardiographic model. Based on the final model a 12-point risk score was derived, indicating the association with the primary outcome within five years. During a median follow-up time of 4.2 years 74 patients died or underwent lung transplantation. The conventional model resulted in a C-Index of 0.539, whereas the extended echocardiographic model improved the discrimination (C-index 0.639, p-value 0.017). Ultimately, the newly developed risk score included WHO functional class, 6-min walking distance, N-terminal brain natriuretic peptide concentrations, pericardial effusion, right atrial area, tricuspid annular plane systolic excursion, and fractional area change. Conclusion: Integrating right heart function assessed by echocardiography improves prediction of death or lung transplantation in PAH patients. Independent validation of this finding is warranted.<\/jats:p>","DOI":"10.3390\/jcm10215130","type":"journal-article","created":{"date-parts":[[2021,11,1]],"date-time":"2021-11-01T22:21:34Z","timestamp":1635805294000},"page":"5130","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Non-Invasive Risk Prediction Based on Right Ventricular Function in Patients with Pulmonary Arterial Hypertension"],"prefix":"10.3390","volume":"10","author":[{"given":"Vazhma","family":"Qaderi","sequence":"first","affiliation":[{"name":"Department of Cardiology, University Heart & Vascular Centre Hamburg, 20246 Hamburg, Germany"},{"name":"Department of Epidemiology and Preventive Medicine, School of Public Health and Preventive Medicine, Monash University, Melbourne, VIC 3004, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jessica","family":"Weimann","sequence":"additional","affiliation":[{"name":"Department of Cardiology, University Heart & Vascular Centre Hamburg, 20246 Hamburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lars","family":"Harbaum","sequence":"additional","affiliation":[{"name":"Department of Pulmonology, University Medical Centre Hamburg-Eppendorf, 20246 Hamburg, Germany"},{"name":"Centre for Pulmonary Arterial Hypertension Hamburg, Martin Zeitz Centre for Rare Diseases, University Medical Centre Hamburg-Eppendorf, 20246 Hamburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benedikt N.","family":"Schrage","sequence":"additional","affiliation":[{"name":"Department of Cardiology, University Heart & Vascular Centre Hamburg, 20246 Hamburg, Germany"},{"name":"German Centre for Cardiovascular Research (DZHK), Partner Site Hamburg\/Kiel\/Luebeck, 20246 Hamburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dorit","family":"Knappe","sequence":"additional","affiliation":[{"name":"Department of Cardiology, University Heart & Vascular Centre Hamburg, 20246 Hamburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7784-8267","authenticated-orcid":false,"given":"Jan K.","family":"Hennigs","sequence":"additional","affiliation":[{"name":"Department of Pulmonology, University Medical Centre Hamburg-Eppendorf, 20246 Hamburg, Germany"},{"name":"Centre for Pulmonary Arterial Hypertension Hamburg, Martin Zeitz Centre for Rare Diseases, University Medical Centre Hamburg-Eppendorf, 20246 Hamburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christoph","family":"Sinning","sequence":"additional","affiliation":[{"name":"Department of Cardiology, University Heart & Vascular Centre Hamburg, 20246 Hamburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7170-9509","authenticated-orcid":false,"given":"Renate B.","family":"Schnabel","sequence":"additional","affiliation":[{"name":"Department of Cardiology, University Heart & Vascular Centre Hamburg, 20246 Hamburg, Germany"},{"name":"German Centre for Cardiovascular Research (DZHK), Partner Site Hamburg\/Kiel\/Luebeck, 20246 Hamburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefan","family":"Blankenberg","sequence":"additional","affiliation":[{"name":"Department of Cardiology, University Heart & Vascular Centre Hamburg, 20246 Hamburg, Germany"},{"name":"German Centre for Cardiovascular Research (DZHK), Partner Site Hamburg\/Kiel\/Luebeck, 20246 Hamburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1881-0197","authenticated-orcid":false,"given":"Paulus","family":"Kirchhof","sequence":"additional","affiliation":[{"name":"Department of Cardiology, University Heart & Vascular Centre Hamburg, 20246 Hamburg, Germany"},{"name":"German Centre for Cardiovascular Research (DZHK), Partner Site Hamburg\/Kiel\/Luebeck, 20246 Hamburg, Germany"},{"name":"Institute of Cardiovascular Sciences, University of Birmingham, Birmingham B15 2TT, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hans","family":"Klose","sequence":"additional","affiliation":[{"name":"Department of Pulmonology, University Medical Centre Hamburg-Eppendorf, 20246 Hamburg, Germany"},{"name":"Centre for Pulmonary Arterial Hypertension Hamburg, Martin Zeitz Centre for Rare Diseases, University Medical Centre Hamburg-Eppendorf, 20246 Hamburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5102-0955","authenticated-orcid":false,"given":"Christina","family":"Magnussen","sequence":"additional","affiliation":[{"name":"Department of Cardiology, University Heart & Vascular Centre Hamburg, 20246 Hamburg, Germany"},{"name":"German Centre for Cardiovascular Research (DZHK), Partner Site Hamburg\/Kiel\/Luebeck, 20246 Hamburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,10,31]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1093\/eurheartj\/ehv317","article-title":"2015 ESC\/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and 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