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Refined risk stratification in pulmonary arterial hypertension and timing of lung transplantation

Karen M Olsson, Manuel J Richter, Jan C Kamp, Henning Gall, Hossein-Ardeshir Ghofrani, Jan Fuge, Ralf Ewert, Marius M Hoeper
European Respiratory Journal 2022; DOI: 10.1183/13993003.03087-2021
Karen M Olsson
1Department of Pneumology, Hannover Medical School, Member of the German Center for Lung Research (DZL)
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Manuel J Richter
2Department of Pneumology, Kerckhoff Heart, Rheuma and Thoracic Center, Bad Nauheim, Germany
3Department of Internal Medicine, Justus Liebig University Giessen, Universities of Giessen and Marburg Lung Center (UGMLC), Giessen, Germany
4Member of the German Center for Lung Research (DZL), Giessen, Germany
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Jan C Kamp
1Department of Pneumology, Hannover Medical School, Member of the German Center for Lung Research (DZL)
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Henning Gall
2Department of Pneumology, Kerckhoff Heart, Rheuma and Thoracic Center, Bad Nauheim, Germany
3Department of Internal Medicine, Justus Liebig University Giessen, Universities of Giessen and Marburg Lung Center (UGMLC), Giessen, Germany
4Member of the German Center for Lung Research (DZL), Giessen, Germany
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Hossein-Ardeshir Ghofrani
2Department of Pneumology, Kerckhoff Heart, Rheuma and Thoracic Center, Bad Nauheim, Germany
3Department of Internal Medicine, Justus Liebig University Giessen, Universities of Giessen and Marburg Lung Center (UGMLC), Giessen, Germany
4Member of the German Center for Lung Research (DZL), Giessen, Germany
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Jan Fuge
1Department of Pneumology, Hannover Medical School, Member of the German Center for Lung Research (DZL)
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Ralf Ewert
5Department of Internal Medicine, Ernst-Moritz-Arndt University, Greifswald, Germany
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Marius M Hoeper
1Department of Pneumology, Hannover Medical School, Member of the German Center for Lung Research (DZL)
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  • For correspondence: olsson.karen@mh-hannover.de
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Extract

In patients with pulmonary arterial hypertension (PAH) presenting with progressive disease despite optimised medical therapy, lung transplantation remains an important treatment option [1–3]. Current guidelines recommend assessing the individual mortality risk regularly using a 3-strata model to determine whether patients are at low, intermediate, or high risk of death [1, 2]. In patients who do not meet low risk criteria with optimised medical therapy, evaluation for lung transplantation should be considered, especially when they require intravenous or subcutaneous therapy with prostacyclin analogues [3]. It is, however, less clear when eligible patients should be actively listed for transplant. In patients who are at high risk of death despite optimised PAH therapy, the 1-year mortality is >20%, i.e., considerably higher than the 1-year mortality after lung transplantation, which is approximately 10% [4, 5]. Hence, high-risk patients have a better chance of survival with transplantation than without. However, no explicit recommendation can be made for patients with an intermediate risk of death while receiving PAH therapy, which can be associated with 1-year mortality rates ranging from 8% to 20% [6, 7].

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: Karen M. Olsson has received fees for lectures and/or consultations from Acceleron, Actelion, Bayer, GSK, Janssen, MSD, Pfizer, and United Therapeutics.

Conflict of interest: Manuel Richter has received honoraria from Actelion, Bayer and Janssen.

Conflict of interest: Jan Kamp has nothing to disclose.

Conflict of interest: Henning Gall reports personal fees from Actelion, AstraZeneca, Bayer, BMS, GSK, Janssen-Cilag, Lilly, MSD, Novartis, OMT, Pfizer and United Therapeutics.

Conflict of interest: H. Ardeshir Ghofrani has received honorariums for consultations and/or speaking at conferences from Bayer HealthCare AG, Actelion, Encysive, Pfizer, Ergonex, Lilly, and Novartis. He is member of advisory boards for Acceleron, Bayer HealthCare AG, Pfizer, GSK, Actelion, Lilly, Merck, Encysive, and Ergonex. He has also received governmental grants from the German Research Foundation (DFG), Excellence Cluster Cardiopulmonary Research (ECCPS), State Government of Hessen (LOEWE), and the German Ministry for Education and Research (BMBF).

Conflict of interest: Jan Fuge has nothing to disclose.

Conflict of interest: Ralf Ewert has received speaker fees and honoraria for consultations from Actelion, Bayer, GSK, Janssen, Lilly, MSD, Novartis, Pfizer, and United Therapeutics.

Conflict of interest: Marius M. Hoeper has received fees for lectures and/or consultations from Acceleron, Actelion, Bayer, GSK, Janssen, MSD, and Pfizer.

  • Received December 5, 2021.
  • Accepted January 29, 2022.
  • Copyright ©The authors 2022. For reproduction rights and permissions contact permissions{at}ersnet.org
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Refined risk stratification in pulmonary arterial hypertension and timing of lung transplantation
Karen M Olsson, Manuel J Richter, Jan C Kamp, Henning Gall, Hossein-Ardeshir Ghofrani, Jan Fuge, Ralf Ewert, Marius M Hoeper
European Respiratory Journal Jan 2022, 2103087; DOI: 10.1183/13993003.03087-2021

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Refined risk stratification in pulmonary arterial hypertension and timing of lung transplantation
Karen M Olsson, Manuel J Richter, Jan C Kamp, Henning Gall, Hossein-Ardeshir Ghofrani, Jan Fuge, Ralf Ewert, Marius M Hoeper
European Respiratory Journal Jan 2022, 2103087; DOI: 10.1183/13993003.03087-2021
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