Open Access

ARTICLE

Long-Term Survival Following the Fontan Procedure: A Focus on Hepatic Function in a Twenty-Year Single-Center Cohort from Southern China

Xiang Liu^1,2,#, Linjiang Han^1,2,#, Zirui Huang^1,2, Jiazichao Tu^1,2, Ruyue Zhang^1,2, Jimei Chen^1,2,*

1 Department of Cardiac Surgery, Guangdong Cardiovascular Institute, Guangdong Provincial People’s Hospital (Guangdong Academy of Medical Sciences), Southern Medical University, Guangzhou, China
2 Guangdong Provincial Key Laboratory of South China Structural Heart Disease, Guangzhou, China

* Corresponding Author: Jimei Chen. Email:
# The authors contributed equally to this work

Structural and Congenital Heart Disease 2026, 21(4), 2 https://doi.org/10.32604/schd.2026.083722

Received 13 April 2026; Accepted 05 August 2026; Issue published 30 September 2026

Abstract

Background: 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. Methods: 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. Results: 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 (p < 0.05). Furthermore, a high preoperative MELD-XI score (>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 (p < 0.05). Conclusions: 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.

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Supplementary Material

Supplementary Material File

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