%0 Journal Article
%A Nosotti, Mario
%A D'Ovidio, Frank
%A Leiva-Juarez, Miguel
%A Keshavjee, Shaf
%A Rackauskas, Mindaugas
%A Van Raemdonck, Dirk
%A Ceulemans, Laurens J
%A Krueger, Thorsten
%A Koutsokera, Angela
%A Schiavon, Marco
%A Rea, Federico
%A Iskender, Ilker
%A Moreno, Paula
%A Alvarez, Antonio
%A Luzzi, Luca
%A Paladini, Piero
%A Rosso, Lorenzo
%A Bertani, Alessandro
%A Venuta, Federico
%A Pecoraro, Ylenia
%A Al-Kattan, Khaled
%A Kubisa, Bartosz
%A Inci, Ilhan
%T Rare indications for a lung transplant. A European Society of Thoracic Surgeons survey.
%D 2020
%U http://hdl.handle.net/10668/16423
%X The European Society of Thoracic Surgeons Lung Transplantation Working Group promoted a survey to evaluate overall survival in a large cohort of patients receiving lung transplants for rare pulmonary diseases. We conducted a retrospective multicentre study. The primary end point was overall survival; secondary end points were survival of patients with the most common diagnoses in the context of rare pulmonary diseases and chronic lung allograft dysfunction (CLAD)-free survival. Finally, we analysed risk factors for overall survival and CLAD-free survival. Clinical records of 674 patients were extracted and collected from 13 lung transplant centres; diagnoses included 46 rare pulmonary diseases. Patients were followed for a median of 3.1 years. The median survival after a lung transplant was 8.5 years. The median CLAD-free survival was 8 years. The multivariable analysis for mortality identified CLAD as a strong negative predictor [hazard ratio (HR) 6.73)], whereas induction therapy was a protective factor (HR 0.68). The multivariable analysis for CLAD occurrence identified induction therapy as a protective factor (HR 0.51). When we stratified patients by CLAD occurrence in a Kaplan-Meier plot, the survival curves diverged significantly (log-rank test: P  We observed that overall survival and CLAD-free survival were excellent. We support the practice of allocating lungs to patients with rare pulmonary diseases because a lung transplant is both effective and ethically acceptable.
%K Lung diseases
%K Lung transplant
%K Rare diseases
%K Respiratory insufficiency
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