Outcomes after Lung Transplantation for Cystic Fibrosis: A Single Center Experience

Purpose Lung transplantation plays a major role in the management of cystic fibrosis (CF) patients with advanced lung disease. The improvement of quality of life after lung transplantation has been proven to be the highest in this population. Methods and Materials We did a retrospective review of the CF patients who were transplanted in our center between 1995 and 2012. Results Our cohort included 116 CF patients who underwent bilateral lung transplantation for cystic fibrosis. At the time of transplantation, 53% of patients were female with a mean age of 30,1 years old (16-50). Recipients were found to be colonized with multiple pathogens. The majority of them had a positive sputum culture for Pseudomonas aeruginosa (91%), Stenotrophomonas maltophilia (26%) and Aspergillus Fumigatus (25%). Diabetes was present in 42% of patient prior transplantation with an increase to 88% of patients after surgery. Pulmonary function tests showed an increase in forced expiratory volume (FEV1) from a mean of 0.82 L in pre-transplant to a mean of 2,86 L at one year post-transplantation. Our survival values were 92% at one year, 80% at 5 years and 61% at 10 years. Acute rejection occurred only in 18% of our CF patients proven by transbronchial biopsies at 2, 6,12 weeks and 6 and 12 months. Bronchiolitis obliterans syndrome (BOS) was seen in 12% of patients and it is the major cause of death. Our multivariate analysis did not identify any predictive factor for acute rejection, BOS or mortality in this cohort. Renal failure was a complication seen in 34% of patients but it only rarely required renal replacement therapy (2%). Conclusions In conclusion, lung transplantation offers cystic fibrosis patients with terminal disease an excellent survival outcome although mortality rises with time. In our cohort, no elements could be identified as prognostic factors.