TY  - EJOU
AU  - Liu, Xiang 
AU  - Han, Linjiang 
AU  - Huang, Zirui 
AU  - Tu, Jiazichao 
AU  - Zhang, Ruyue 
AU  - Chen, Jimei 

TI  - Long-Term Survival Following the Fontan Procedure: A Focus on Hepatic Function in a Twenty-Year Single-Center Cohort from Southern China
T2  - Structural and Congenital Heart Disease

PY  - 
VL  - 
IS  - 
SN  - 3071-1738

AB  - <b>Background:</b> This study aimed to evaluate the long-term prognosis and influencing factors in patients who underwent Fontan palliation. We specifically investigated the impact of demographic characteristics, preoperative clinical conditions, surgical techniques, and postoperative variables-particularly liver dysfunction markers such as the Model for End-Stage Liver Disease excluding the international normalized ratio (MELD-XI) score-on survival outcomes. <b>Methods:</b> We conducted a retrospective cohort study involving 322 patients who underwent the Fontan procedure. Comprehensive data on demographics, perioperative parameters, and long-term outcomes were collected. Kaplan-Meier survival analysis was performed to estimate 1-, 5-, 10-, and 15-year survival rates, with subgroup comparisons using log-rank tests. Univariate and multivariate Cox proportional hazards models were used to identify independent predictors of mortality, including sex, anatomical anomalies, hemodynamic indices, vasoactive-inotropic score (VIS), and hepatic fibrosis and injury biomarkers. <b>Results:</b> The 15-year overall survival rate was 83.9%. Male sex, heterotaxy syndrome, and anomalous pulmonary venous return were each significantly associated with worse long-term survival rates (<i>p</i> &lt; 0.05). Furthermore, a high preoperative MELD-XI score (&gt;9.44), measured before Fontan completion as a baseline physiological indicator, was significantly associated with worse long-term survival, and Fontan patients demonstrated markedly elevated plasma levels of hepatic biomarkers, including hyaluronic acid (HA), procollagen type III N-terminal peptide (PIIINP), tissue inhibitor of metalloproteinases-1 (TIMP-1), and microRNA-122 (miR-122), compared to healthy controls. Finally, multivariate analysis revealed that asplenia (hazard ratio [HR] = 5.953), elevated mean pulmonary artery pressure (PAP; HR = 1.127), increased pulmonary vascular resistance (PVR; HR = 1.077), higher 48-h VIS (HR = 1.081), and elevated MELD-XI score (HR = 3.145) were independent predictors of mortality (<i>p</i> &lt; 0.05). <b>Conclusions:</b> Long-term survival after Fontan palliation is generally favorable; however, it is significantly compromised by certain congenital anatomical syndromes and markers of physiological stress. Asplenia, indices of pulmonary hypertension, postoperative hemodynamic support, and liver dysfunction (as measured by the MELD-XI score) serve as robust independent predictors of mortality. These findings underscore the importance of early risk stratification and vigilant monitoring of hepatic function to guide clinical management and potentially improve long-term outcomes.
KW  - Fontan procedure; risk predictors; MELD-XI score; hepatic biomarkers; survival

DO  - 10.32604/schd.2026.083722