Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2025
Characteristics and outcomes of people with cystic fibrosis on the Eurotransplant liver transplantation waiting list.
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van der Spek Marissa I, Verkade Henkjan J, Jonker Johan W, de Meijer Vincent E, Bodewes Frank A J A
Аннотация
BACKGROUND: Advanced cystic fibrosis (CF) liver disease can necessitate liver transplantation. This study aims to investigate characteristics, waiting list dynamics, and waiting list mortality of people with CF (pwCF) registered for liver transplantation within Eurotransplant countries, to understand and improve transplant outcomes for this group.
METHODS: We analysed Eurotransplant liver transplantation registration data (January 2007-December 2022), comparing pwCF to people with no CF (pwnoCF) and non-CF age/liver disease severity score (Lab-MELD) matched controls, with subgroup comparisons between pwCF receiving isolated liver transplantation (LiverTx) and combined liver-lung transplantation (Liver+LungTx).
RESULTS: 215 out of 38,125 liver transplantation registrations were for pwCF. 65.1 % of the pwCF were listed for LiverTx and 34.9 % for Liver+LungTx. At registration, pwCF were younger (17.9 ± 0.8 vs. 55.0 ± 0.1; P < 0.05) and had lower Lab-MELD scores (10.0 ± 0.4 vs. 15.0 ± 0.0; P < 0.05) compared to pwnoCF. PwCF listed for LiverTx were younger (15.1 ± 0.9 vs. 26.0 ± 1.1; P < 0.05) and had higher Lab-MELD scores at transplantation (12.0 ± 1.0 vs. 9.0 ± 0.5; P < 0.05) compared to Liver+LungTx. PwCF had a higher 2-year waiting list mortality than non-CF matched controls (14.9 % vs. 0.0 %; P < 0.05). Within pwCF, mortality was higher for pwCF listed for Liver+LungTx than for LiverTx (30.7 % vs. 6.4 %; P < 0.05).
CONCLUSIONS: CF represents a distinct indication for liver transplantation, with increased varying waiting list mortality risks across the different CF-groups, not adequately captured by standard liver disease severity scores. PwCF considered for liver transplantation require close monitoring as their disease severity and mortality risk are not well represented in the current allocation algorithm, necessitating adaptations to the organ allocation rules.
METHODS: We analysed Eurotransplant liver transplantation registration data (January 2007-December 2022), comparing pwCF to people with no CF (pwnoCF) and non-CF age/liver disease severity score (Lab-MELD) matched controls, with subgroup comparisons between pwCF receiving isolated liver transplantation (LiverTx) and combined liver-lung transplantation (Liver+LungTx).
RESULTS: 215 out of 38,125 liver transplantation registrations were for pwCF. 65.1 % of the pwCF were listed for LiverTx and 34.9 % for Liver+LungTx. At registration, pwCF were younger (17.9 ± 0.8 vs. 55.0 ± 0.1; P < 0.05) and had lower Lab-MELD scores (10.0 ± 0.4 vs. 15.0 ± 0.0; P < 0.05) compared to pwnoCF. PwCF listed for LiverTx were younger (15.1 ± 0.9 vs. 26.0 ± 1.1; P < 0.05) and had higher Lab-MELD scores at transplantation (12.0 ± 1.0 vs. 9.0 ± 0.5; P < 0.05) compared to Liver+LungTx. PwCF had a higher 2-year waiting list mortality than non-CF matched controls (14.9 % vs. 0.0 %; P < 0.05). Within pwCF, mortality was higher for pwCF listed for Liver+LungTx than for LiverTx (30.7 % vs. 6.4 %; P < 0.05).
CONCLUSIONS: CF represents a distinct indication for liver transplantation, with increased varying waiting list mortality risks across the different CF-groups, not adequately captured by standard liver disease severity scores. PwCF considered for liver transplantation require close monitoring as their disease severity and mortality risk are not well represented in the current allocation algorithm, necessitating adaptations to the organ allocation rules.