Prognostic impact of liver function in cystic fibrosis patients undergoing lung transplantation

Aims: Cystic fibrosis (CF) is a life-threatening autosomal recessive hereditary disease that impacts the function of multiple organ systems. In end stage disease lung transplantation has become a viable option to improve the quality of life and prolong survival. CF liver disease (CFLD) as comorbidity develops in 8–17% of CF patients with unknown impact on prognosis of lung transplantation. Methods: 31 patients with cystic fibrosis who underwent double lung transplantation (DLTx) from 1999 to 2009 in our clinic were included in the retrospective study. Posttransplant survival was assessed and liver parameters (AST, ALT, GGT, bilirubin), hemoglobin, leucocytes, INR, creatinin and the MELD-Score were determined preoperatively as well as one day and 4 weeks postoperatively. The prognostic impact of liver function on patient outcome was analysed. Results: Patients had a mean age of 25 (15–38) and posttransplant survival was 74% after 1y, 71% after 3y, and 68% after 5y. Overall liver serum parameters were within standard ranges pre- and postoperatively. Reduced serum concentrations of hemoglobin and elevated leucocytes as well as CRP were found. Patients were then grouped according to post transplant survival, with death in the first year (8 patients, group I) and long term survival (23 patients, group II). Group I had significantly higher MELD preoperatively and significantly increased bilirubin and MELD postoperatively compared to group II. Moreover single patient survival analysis revealed that those with elevated GGT had a significant higher risk of dying. Conclusion: Liver function parameters and probably the already established MELD-Score could be utilized to predict outcome of patients receiving DLTx for CF. Evaluation of the prognostic value should be confirmed in larger prospective studies.