The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation · 2026
Heart Transplantation in Adult Fontan Patients: New Insights from Fontan-Associated Liver Disease Beyond Traditional Predictors.
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Rybicka Justyna, Coats Louise, Crossland David, Patel Preya, Scott John, Anderson Kirsty, Hermuzi Antony, Adhvaryu Kaitav, Kakarla Jayant, Erturk Emre, Seller Neil, White Steven, Gonzalez-Fernandez Oscar, MacGowan Guy, Nassar Mohamed S, Kenny Louise, Jansen Katrijn
Аннотация
BACKGROUND: Fontan patients are considered high risk for heart transplantation (HT) owing to their complex anatomy, multiple prior chest entries, and universal Fontan-associated liver disease (FALD). The impact of FALD and other preoperative factors remains, however, uncertain.
OBJECTIVES: We sought to identify factors associated with 90-day mortality after isolated HT in adults with Fontan circulation, with particular focus on the relationship between liver-related parameters and post-transplant outcomes.
METHODS: A retrospective cohort study of all adult Fontan patients undergoing isolated HT was conducted. Factors associated with 90-day mortality were assessed using appropriate univariate analyses and penalized multivariable logistic regression. ROC analysis was used to derive liver size cut-offs and compare the discriminative performance of selected variables. A descriptive analysis of adult Fontan patients undergoing combined heart-liver transplantation (CHLT) was also performed for contextual comparison.
RESULTS: Thirty-seven patients (mean age 30 years) underwent isolated HT; 19% (n=8) died within 90 days. Factors associated with increased 90-day mortality were >4 previous chest entries, major perioperative bleeding, and small liver size [total liver volume (TLV) ≤1366 mL, total liver volume indexed to body surface area (TLVi) <786 mL/m2 and liver transverse dimension (LTD) <21 cm]. Multiple chest veno-venous collaterals, >4 previous chest entries, and small liver were strongly associated with bleeding and subsequent fatal complications including sepsis, graft dysfunction, and bowel ischaemia. Within the study period, five other patients had undergone CHLT. All had biopsy confirmed advanced liver disease with features of clinically significant portal hypertension or hepatocellular carcinoma. One patient died within 90 days following prolonged cardiopulmonary bypass, major bleeding, graft dysfunction and sepsis, culminating in multiorgan failure.
CONCLUSIONS: In Fontan patients undergoing isolated HT, >4 previous chest entries, major bleeding, and small liver volume were associated with increased 90-day mortality. Objective liver metrics (TLV ≤1366 mL, TLVi <786 mL/m2, TLD <21 cm) may provide adjunctive risk stratification for Fontan patients considered for isolated HT; however, these measures and proposed thresholds remain exploratory and require external validation. CHLT is a viable option, although non-liver related transplant risk factors may similarly influence outcomes. Detailed liver assessment and management at experienced, high-volume congenital transplant centres are important to optimizing outcomes in this complex population.
OBJECTIVES: We sought to identify factors associated with 90-day mortality after isolated HT in adults with Fontan circulation, with particular focus on the relationship between liver-related parameters and post-transplant outcomes.
METHODS: A retrospective cohort study of all adult Fontan patients undergoing isolated HT was conducted. Factors associated with 90-day mortality were assessed using appropriate univariate analyses and penalized multivariable logistic regression. ROC analysis was used to derive liver size cut-offs and compare the discriminative performance of selected variables. A descriptive analysis of adult Fontan patients undergoing combined heart-liver transplantation (CHLT) was also performed for contextual comparison.
RESULTS: Thirty-seven patients (mean age 30 years) underwent isolated HT; 19% (n=8) died within 90 days. Factors associated with increased 90-day mortality were >4 previous chest entries, major perioperative bleeding, and small liver size [total liver volume (TLV) ≤1366 mL, total liver volume indexed to body surface area (TLVi) <786 mL/m2 and liver transverse dimension (LTD) <21 cm]. Multiple chest veno-venous collaterals, >4 previous chest entries, and small liver were strongly associated with bleeding and subsequent fatal complications including sepsis, graft dysfunction, and bowel ischaemia. Within the study period, five other patients had undergone CHLT. All had biopsy confirmed advanced liver disease with features of clinically significant portal hypertension or hepatocellular carcinoma. One patient died within 90 days following prolonged cardiopulmonary bypass, major bleeding, graft dysfunction and sepsis, culminating in multiorgan failure.
CONCLUSIONS: In Fontan patients undergoing isolated HT, >4 previous chest entries, major bleeding, and small liver volume were associated with increased 90-day mortality. Objective liver metrics (TLV ≤1366 mL, TLVi <786 mL/m2, TLD <21 cm) may provide adjunctive risk stratification for Fontan patients considered for isolated HT; however, these measures and proposed thresholds remain exploratory and require external validation. CHLT is a viable option, although non-liver related transplant risk factors may similarly influence outcomes. Detailed liver assessment and management at experienced, high-volume congenital transplant centres are important to optimizing outcomes in this complex population.