Liver Transplantation in Patients With Cystic Fibrosis: 30 Years of Experience in Australia and New Zealand.

OBJECTIVES

To review the waiting list data and outcomes after liver transplantation (LT) in patients with cystic fibrosis (CF) in Australia and New Zealand.

METHODS

The Australia and New Zealand Liver and Intestinal Transplant Registry was utilised to identify patients with CF listed for LT, including as a component of multi-organ transplantation. Outcomes were compared to non-CF patients.

RESULTS

Between January 1989 and July 2022, 55 patients with CF underwent LT at a median age of 18.9 (13.5-30.0) years. Twenty-five (45%) were less than 18 years of age at the time of LT. Thirty-seven (67%) underwent isolated LT, and 18 (33%) underwent LT as a component of multi-organ transplantation. In paediatric CF isolated LT recipients, 15-year survival was similar to non-CF recipients (90.9% vs. 83.4%, p = 0.71); however, waiting list mortality was higher (3/21 [14.3%] vs. 33/779 [4.2%], p = 0.03). In adult CF recipients, there was no significant difference in 5- and 10-year survival after isolated LT (CF: 72.7% and 60.6% vs. non-CF: 83.0% and 73.0%, respectively, p = 0.07), and no significant difference in 5- and 10-year survival after multi-organ transplantation (excluding liver-kidney) compared to non-CF recipients (CF: 60.7% and 45.5% vs. non-CF: 66.7% and 66.7%, p = 0.27). In adult CF recipients, waiting time was longer (293 days [IQR 69-534] vs. 82 days [IQR 19-203], p = 0.004), and time to waiting list mortality in those listed for multi-organ transplantation was shorter (129 days [IQR 47-161] vs. 351 days [IQR 166-691], p = 0.049) than non-CF recipients.

CONCLUSIONS

Survival after isolated LT in children with CF is equivalent to non-CF patients; however, waiting list mortality is higher. Adults with CF experience longer waiting times and shorter time to waiting list mortality.