TY - JOUR
T1 - Morbidity and Mortality in Adult Fontan Patients After Heart or Combined Heart-Liver Transplantation
AU - Lewis, Matthew J.
AU - Reardon, Leigh C.
AU - Aboulhosn, Jamil
AU - Haeffele, Christiane
AU - Chen, Sharon
AU - Kim, Yuli
AU - Fuller, Stephanie
AU - Forbess, Lisa
AU - Alshawabkeh, Laith
AU - Urey, Marcus A.
AU - Book, Wendy M.
AU - Rodriguez, Fred
AU - Menachem, Jonathan N.
AU - Clark, Daniel E.
AU - Valente, Anne Marie
AU - Carazo, Matthew
AU - Egbe, Alexander
AU - Connolly, Heidi M.
AU - Krieger, Eric V.
AU - Angiulo, Jilian
AU - Cedars, Ari
AU - Ko, Jong
AU - Jacobsen, Roni M.
AU - Earing, Michael G.
AU - Cramer, Jonathan W.
AU - Ermis, Peter
AU - Broda, Christopher
AU - Nugaeva, Natalia
AU - Ross, Heather
AU - Awerbach, Jordan D.
AU - Krasuski, Richard A.
AU - Rosenbaum, Marlon
N1 - Publisher Copyright:
© 2023 American College of Cardiology Foundation
PY - 2023/6/6
Y1 - 2023/6/6
N2 - Background: An increasing number of adult Fontan patients require heart transplantation (HT) or combined heart-liver transplant (CHLT); however, data regarding outcomes and optimal referral time remain limited. Objectives: The purpose of this study was to define survivorship post-HT/CHLT and predictors of post-transplant mortality, including timing of referral, in the adult Fontan population. Methods: A retrospective cohort study of adult Fontan patients who underwent HT or CHLT across 15 centers in the United States and Canada was performed. Inclusion criteria included the following: 1) Fontan; 2) HT/CHLT referral; and 3) age ≥16 years at the time of referral. Date of “failing” Fontan was defined as the earliest of the following: worsening fluid retention, new ascites, refractory arrhythmia, “failing Fontan” diagnosis by treating cardiologist, or admission for heart failure. Results: A total of 131 patients underwent transplant, including 40 CHLT, from 1995 to 2021 with a median post-transplant follow-up time of 1.6 years (Q1 0.35 years, Q3 4.3 years). Survival was 79% at 1 year and 66% at 5 years. Survival differed by decade of transplantation and was 87% at 1 year and 76% at 5 years after 2010. Time from Fontan failure to evaluation (HR/year: 1.23 [95% CI: 1.11-1.36]; P < 0.001) and markers of failure, including NYHA functional class IV (HR: 2.29 [95% CI: 1.10-5.28]; P = 0.050), lower extremity varicosities (HR: 3.92 [95% CI: 1.68-9.14]; P = 0.002), and venovenous collaterals (HR: 2.70 [95% CI: 1.17-6.20]; P = 0.019), were associated with decreased post-transplant survival at 1 year in a bivariate model that included transplant decade. Conclusions: In our multicenter cohort, post-transplant survival improved over time. Late referral after Fontan failure and markers of failing Fontan physiology, including worse functional status, lower extremity varicosities, and venovenous collaterals, were associated with post-transplant mortality.
AB - Background: An increasing number of adult Fontan patients require heart transplantation (HT) or combined heart-liver transplant (CHLT); however, data regarding outcomes and optimal referral time remain limited. Objectives: The purpose of this study was to define survivorship post-HT/CHLT and predictors of post-transplant mortality, including timing of referral, in the adult Fontan population. Methods: A retrospective cohort study of adult Fontan patients who underwent HT or CHLT across 15 centers in the United States and Canada was performed. Inclusion criteria included the following: 1) Fontan; 2) HT/CHLT referral; and 3) age ≥16 years at the time of referral. Date of “failing” Fontan was defined as the earliest of the following: worsening fluid retention, new ascites, refractory arrhythmia, “failing Fontan” diagnosis by treating cardiologist, or admission for heart failure. Results: A total of 131 patients underwent transplant, including 40 CHLT, from 1995 to 2021 with a median post-transplant follow-up time of 1.6 years (Q1 0.35 years, Q3 4.3 years). Survival was 79% at 1 year and 66% at 5 years. Survival differed by decade of transplantation and was 87% at 1 year and 76% at 5 years after 2010. Time from Fontan failure to evaluation (HR/year: 1.23 [95% CI: 1.11-1.36]; P < 0.001) and markers of failure, including NYHA functional class IV (HR: 2.29 [95% CI: 1.10-5.28]; P = 0.050), lower extremity varicosities (HR: 3.92 [95% CI: 1.68-9.14]; P = 0.002), and venovenous collaterals (HR: 2.70 [95% CI: 1.17-6.20]; P = 0.019), were associated with decreased post-transplant survival at 1 year in a bivariate model that included transplant decade. Conclusions: In our multicenter cohort, post-transplant survival improved over time. Late referral after Fontan failure and markers of failing Fontan physiology, including worse functional status, lower extremity varicosities, and venovenous collaterals, were associated with post-transplant mortality.
KW - adult Fontan
KW - combined heart-liver transplant
KW - Fontan-associated liver disease
KW - heart transplant
KW - referral timing
UR - https://www.scopus.com/pages/publications/85159692564
UR - https://www.scopus.com/pages/publications/85159692564#tab=citedBy
U2 - 10.1016/j.jacc.2023.03.422
DO - 10.1016/j.jacc.2023.03.422
M3 - Article
C2 - 37257951
AN - SCOPUS:85159692564
SN - 0735-1097
VL - 81
SP - 2161
EP - 2171
JO - Journal of the American College of Cardiology
JF - Journal of the American College of Cardiology
IS - 22
ER -