CYSTIC FIBROSIS

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

G Meachery¹, A De Soyza², A Nicholson³, G Parry¹, A Hasan¹, K Tocewicz¹, T Pillay¹, S Clark¹, J L Lordan^1,2, S Schueler¹, A J Fisher^1,2, J H Dark¹, F K Gould³ and P A Corris^1,2

¹ Department of Cardiopulmonary Transplantation, The Freeman Hospital, High Heaton, Newcastle-upon-Tyne, UK
² Applied Immunobiology and Transplantation, Institute of Cellular Medicine, University of Newcastle, UK
³ Department of Medical Microbiology, The Freeman Hospital, High Heaton, Newcastle-upon-Tyne, UK

Correspondence to:
Dr A De Soyza, Applied Immunobiology and Transplantation, Institute of Cellular Medicine, University of Newcastle, Newcastle-upon-Tyne NE7 7DN, UK; anthony.de-soyza{at}ncl.ac.uk

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.

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