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

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.

Risk of Post-Lung Transplant Renal Dysfunction in Adults With Cystic Fibrosis

“…[6][7][8] In the overall lung transplant population, the cumulative incidence of posttransplant renal dysfunction has been reported to range between 4% and 35%, depending on how renal dysfunction is defi ned and the severity of renal dysfunction. 1,[9][10][11][12] Renal dysfunction complicates posttransplant management, as it usually prompts a reduction in the dosing of immunosuppressive medications or, if severe enough, necessitates a change in the immunosuppressive regimen. 7 It can also progress to require dialysis and/or kidney transplantation.…”

“…2,8 To our knowledge, no previous studies have looked at the incidence or predictors of posttransplantation renal dysfunction in the CF population. Previous studies of post-lung transplant renal dysfunction have been single center 10 and/or have not looked specifically at the CF population. 8,9,11 We suspect individuals with CF might be at heightened risk for developing renal dysfunction post-lung transplant because of increased risk for dehydration 14 and repeated exposure to nephrotoxic antibiotics used to treat chronic pulmonary infections.…”

El trasplante pulmonar en la fibrosis quística y la influencia de la circulación extracorpórea

“…Los autores mencionan también que posterior al TP, 20 % de los pacientes fueron diagnosticados de DM. 70 Lo que representa que aparte de que una gran proporción de pacientes ya son diabéticos al momento del TP, durante el postoperatorio, un gran porcentaje se volverá diabético.…”

“…257 Meachery y cols en su serie de 176 pacientes FQ que recibieron un TP, no encuentran una mayor mortalidad en los pacientes portadores de VMNI (p = 0,89). 70 Al igual que las publicaciones previas, nuestra serie no encuentra un aumento de mortalidad en los pacientes que requirieron VMNI previa al TP (p = 1,00). En nuestra serie, la media de metros caminados en el 6WT fue de 323, resultado muy similar al de otra serie nacional.…”

“…54 La supervivencia libre de RC se presenta en la literatura del 85 % al año, 74 % a los 5 años y del 38% a los 10 años del TP. 70 La frecuencia de RA y RC de nuestra serie es mayor a la presentada en la literatura. Un alto porcentaje de nuestros pacientes fueron diagnosticados por BTB.…”