Rare indications for a lung transplant. A European Society of Thoracic Surgeons survey. (15th October 2020)

• Record Type: Journal Article
• Authors: 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
• Is Part Of: Interactive cardiovascular and thoracic surgery. Volume 31:Number 5(2020)
• Page Start: 638
• Page End: 643
• Publication Date: 2020-10-15
• Subjects: Lung transplant -- Rare diseases -- Lung diseases -- Respiratory insufficiency; Chest -- Surgery -- Periodicals; Cardiovascular system -- Diseases -- Periodicals; 616.1
• DOI: 10.1093/icvts/ivaa165
• Languages: English
• ISSNs: 1569-9293

Abstract

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 ethically acceptable. Abstract : A lung transplant is the only treatment available for selected patients with end-stage respiratory insufficiency.