Clinical Features and Outcomes of Cystic Fibrosis related Liver Transplantation in a National Transplant Centre

<bold>Background:</bold> Cystic Fibrosis(CF) related liver disease(CFLD) defines a spectrum of liver involvement that occurs in 30-50% of patients with CF.CF patients with end stage liver disease should be considered for liver transplantation. <bold>Methods:</bold> Retrospective review from a national liver and pancreas transplant centre between 2009-2024 looking at patient features and outcomes from liver transplantation in CFLD. <bold>Results:</bold> 9 patients had liver transplantation between 2009-2019 for CFLD. 56%(n=5)of these had the δF508 homozygous CF mutation.77%(n=7) of the patients had non cirrhotic CFLD. Mean MELD score prior to transplant was 19 giving a 3-4% predicted 90-day mortality risk.78%(n=7) had a Child-Pugh score of C giving a predicted life expectancy of 1-3 years without transplant.1 year survival of the transplanted patients was 88%(n=8),5-year survival was 78%(n=7) and 10-year survival was 67%(n=6). Excluding the patient who had a combined liver-lung transplant mean FEV1 pre- transplant was 1.78L and 1.83L post-transplant giving a mean improvement of 0.05L(p>0.05). Among the transplanted cohort currently alive 67%(n=4) are managed with CFTR modulator therapy with Kaftrio. 50%(n=2) of these patients have transaminases > 3x upper limit of normal post tranplant but this is biopsy proven not drug effect related. <bold>Conclusions:</bold> Liver Transplantation for CFLD has similar outcomes to transplantation for other forms of liver disease and appears to have a mortality risk benefit. Improvements in FEV1 post-transplant may be due to a reduction in pulmonary complications of advanced CFLD or due to patients being commenced on CFTR modulators post-transplant. Kaftrio appears to be safe post liver transplant.