OBJECTIVES: 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.METHODS: 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.RESULTS: 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 < 0.001). Patients with rare diseases who received transplants had chronic rejection rates similar to those 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 to patients with rare pulmonary diseases because a lung transplant is both effective and acceptable.
Rare indications for a lung transplant. A European Society of Thoracic Surgeons survey / Nosotti, Mario; D'Ovidio, Frank; Leiva-Juarez, Miguel; Keshavjee, Shaf; Rackauskas, Mindaugas; Van Raemdonck, Dirk; Ceulemans, Laurens J; Krueger, Thorsten; Koutsokera, Angela; Schiavon, Marco; Rea, Federico; Iskender, Ilker; Moreno, Paula; Alvarez, Antonio; Luzzi, Luca; Paladini, Piero; Rosso, Lorenzo; Bertani, Alessandro; Venuta, Federico; Pecoraro, Ylenia; Al-Kattan, Khaled; Kubisa, Bartosz; Inci, Ilhan. - In: INTERACTIVE CARDIOVASCULAR AND THORACIC SURGERY. - ISSN 1569-9293. - 31:5(2020), pp. 638-643. [10.1093/icvts/ivaa165]
Rare indications for a lung transplant. A European Society of Thoracic Surgeons survey
Venuta, Federico;Pecoraro, Ylenia;
2020
Abstract
OBJECTIVES: 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.METHODS: 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.RESULTS: 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 < 0.001). Patients with rare diseases who received transplants had chronic rejection rates similar to those 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 to patients with rare pulmonary diseases because a lung transplant is both effective and acceptable.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.