ANZ journal of surgery · 2026
Liver Transplantation in Patients With Cystic Fibrosis: 30 Years of Experience in Australia and New Zealand.
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Wilson Thomas G, Fink Michael, Illhardt Toni, Jones Robert M, Majumdar Avik, Byrne Mandy, Oliver Mark R
Аннотация
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.
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.