Rare indications for a lung transplant. A European Society of Thoracic Surgeons Survey

OBJECTIVES:The European Society of Thoracic Surgeons Lung Transplantation Working Group promoted a survey to evaluate overall sur-vival in a large cohort of patients receiving lung transplants for rare pulmonary diseases.METHODS:We conducted a retrospective multicentre study. The primary end point was overall survival; secondary end points were sur-vival 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.RESULTS:Clinical records of 674 patients were extracted and collected from 13 lung transplant centres; diagnoses included 46 rare pulmo-nary diseases. Patients were followed for a median of 3.1 years. The median survival after a lung transplant was 8.5 years. The medianCLAD-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 inductiontherapy as a protective factor (HR 0.51). When we stratified patients by CLAD occurrence in a Kaplan–Meier plot, the survival curves di-verged significantly (log-rank test:P< 0.001). Patients with rare diseases who received transplants had chronic rejection rates similar tothose of the general population who received transplants.CONCLUSIONS:We observed that overall survival and CLAD-free survival were excellent. We support the practice of allocating lungs topatients with rare pulmonary diseases because a lung transplant is both effective and ethically acceptable.