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
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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