Outcomes of lung transplantation for cystic fibrosis in a large UK cohort

Gerard Meachery, Anthony de Soyza, Audrey Nicholson, Gareth Parry, Asif Hasan, Krzysztof Tocewicz, Thasee Pillay, Stephen Clark, James Lordan, Stephan Schueler, Andrew Fisher, John Dark, Frances Kate Gould, Paul Corris

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99 Citations (Scopus)

Abstract

Background: Lung transplantation is an important option to treat patients with advanced cystic fibrosis (CF) lung disease. The outcomes of a large UK cohort of CF lung transplantation recipients is reported.Methods: Retrospective review of case notes and transplantation databases.Results: 176 patients with CF underwent lung transplantation at our centre. The majority (168) had bilateral sequential lung transplantation. Median age at transplantation was 26 years. Diabetes was common pretransplantation (40%). Polymicrobial infection was common in individual recipients. A diverse range of pathogens were encountered, including the Burkholderia cepacia complex (BCC). The bronchial anastomotic complication rate was 2%. Pulmonary function (forced expiratory volume in 1 s % predicted) improved from a pretransplantation median of 0.8 l (21% predicted) to 2.95 l (78% predicted) at 1 year following transplantation. We noted an acute rejection rate of 41% within the first month. Our survival values were 82% survival at 1 year, 70% at 3 years, 62% at 5 years and 51% at 10 years. Patients with BCC infection had poorer outcomes and represented the majority of those who had a septic death. Data are presented on those free from these infections. Bronchiolitis obliterans syndrome (BOS) and sepsis were common causes of death. Freedom from BOS was 74% at 5 years and 38% at 10 years. Biochemical evidence of renal dysfunction was common although renal replacement was infrequently required (<5%). Conclusion: Lung transplantation is an important therapeutic option in patients with CF even in those with more complex microbiology. Good functional outcomes are noted although transplantation associated morbidities accrue with time.
Original languageEnglish
Pages (from-to)725-731
JournalThorax
Volume63
Issue number8
DOIs
Publication statusPublished - 2008

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