Cystic fibrosis is a genetic multi-system disease affecting approximately 1 in every 2500 newborns globally. Pulmonary transplantation is indicated in patients with severe bronchiectasis and hepatic cirrhosis occurs in about 15% of CF patients starting as early as in the first decade of life.

There are limited reports in the literature about recipients of combined liver and lung transplantation. Most reports are from a single center experience and for CF patients alone. This report examines cases of combined liver and lung transplantation reported in the Scientific Registry of Transplant Recipients (SRTR) database, merging the lung and liver databases using unique identifiers to increase the available variables for investigation. 73 patients received a combined liver and lung transplantation from the period of 1994-2015. Our objective was to compare the outcomes of combined lung and liver transplantation in the Scientific Registry of Transplant Recipients (SRTR) for patients transplanted for Cystic Fibrosis vs. Other Indication.

Recipients' demographics demonstrated 17 pediatric vs. 56 adult recipients. In the pediatric population, the majority of the children (n=16) were diagnosed with CF and one child was diagnosed with Alagille's syndrome. The mean age for pediatric patents at transplantation was 14. In the adult group, 33 patients had a CF diagnosis with idiopathic fibrosis being the second most common diagnosis (n=8).

In the Adult Liver/Lung recipients, there was a significant difference in the mean age between the CF and non-CF groups, 28 vs 52 respectively (p value 0.001). Weight (71 vs. 55kg) and BMI (24 vs. 19) were also significantly higher in the non-CF groups. Finally, there was a significantly higher incident of Diabetes Mellitus in the CF group (57% vs 12%). For liver transplantation, the most common indication other than CF (n=46) was hepatitis C cirrhosis (n=7).

Overall patient survival rate at 1 year and 5 years was 76% and 61%, respectively. There was no significant difference in survival rate comparing patients with a diagnosis of CF as compared to other indication. Further analysis demonstrated no significant survival difference between the survivals of patients within the different age groups (pediatric vs adult).

CITATION INFORMATION: Halldorson J, AlQahtani K. Outcomes of Combined Liver/Lung Transplantation for Cystic Fibrosis Using SRTR Analysis. Am J Transplant. 2017;17 (suppl 3).

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