Background: Lung transplantation is an important option to treat patients with advanced Cystic Fibrosis (CF) lung disease. We report the outcomes of a large UK cohort of CF lung transplantation recipients.
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 pre-transplantation (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 (FEV1% predicted) improved from pre-transplantation median 0.8 litres (21% predicted) to 2.95 litres (78% predicted) at one year following transplantation. We noted an acute rejection rate of 41% within the first month. Our survival figures were 82% survival at one year, 70% at three years, 62% at five years and 51% at ten years. Patients with BCC infection had poorer outcomes and represented the majority of those who had a septic death. We present data on those free from these infections. Bronchiolitis Obliterans Syndrome (BOS) and sepsis were common causes of death. Freedom from BOS was 74% at five years and 38 % at ten years. Biochemical evidence of renal dysfunction was common though renal replacement was infrequently required (<5%).
Interpretation: 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.