Téllez Luis, Rincón Diego, Payancé Audrey, Jaillais Anaïs, Lebray Pascal, Rodríguez de Santiago Enrique, Clemente Ana, Paradis Valerie, Lefort Bruno, Garrido-Lestache Elvira, Prieto Raquel, Iserin Laurence, Tallegas Matthias, Garrido Elena, Torres María, Muriel Alfonso, Perna Cristian, Del Cerro María Jesús, d'Alteroche Louis, Rautou Pierre-Emmanuel, Bañares Rafael, Albillos Agustín
Gastroenterology and Hepatology Department, Hospital Universitario Ramón y Cajal, IRYCIS, CIBERehd, Universidad de Alcalá, Madrid, Spain.
Liver Unit, Hospital General Universitario Gregorio Marañón, Instituto de Investigación Sanitaria Gregorio Marañón, CIBERehd, Universidad Complutense, Madrid, Spain.
J Hepatol. 2025 Mar;82(3):480-489. doi: 10.1016/j.jhep.2024.09.005. Epub 2024 Sep 10.
BACKGROUND & AIMS: Fontan-type surgery is a palliative procedure for congenital heart disease with univentricular physiology that may, in the long term, lead to advanced chronic liver disease. Herein, we assessed the accuracy of conventional non-invasive models for assessing liver fibrosis in the context of Fontan circulation and developed a new risk score employing non-invasive tools.
A prospective, cross-sectional, observational study was conducted across five European centers and encompassing all consecutive adult patients with Fontan circulation, liver biopsy and non-invasive tests (e.g. elastography, APRI, and FIB-4). The primary outcome was the identification of severe liver fibrosis on biopsy. Multivariable logistic regression identified non-invasive predictors of severe fibrosis, leading to the development and internal validation of a new scoring model named the FonLiver risk score.
In total, 217 patients (mean [SD] age, 27.9 [8.9] years; 50.7% males) were included. Severe liver fibrosis was present in 47.9% (95% CI 41.2%-54.5%) and correlated with a lower functional class, protein-losing enteropathy, and compromised cardiopulmonary and systemic hemodynamics. The final FonLiver risk score incorporated liver stiffness measurement using transient elastography and platelet count, and demonstrated strong discrimination and calibration (AUROC of 0.81). The FonLiver risk score outperformed conventional prediction models (e.g. APRI and FIB-4), which all exhibited worse performance in our cohort (AUROC <0.70 for all).
Severe liver fibrosis is prevalent in adults following Fontan-type surgery and can be effectively estimated using the novel FonLiver risk score. This scoring system can be easily incorporated into the routine assessment of patients with Fontan circulation.
Fontan-type surgery is used as a palliative procedure for congenital heart disease with univentricular physiology that may, in the long term, lead to advanced chronic liver disease. The severity of liver fibrosis progression has been proposed as a surrogate for failing Fontan hemodynamics as well as worse outcomes after heart transplantation. The development of FALD screening protocols would facilitate the early detection of advanced fibrosis and anticipate interventions to optimize the Fontan circulation, thereby improving outcomes. In our international series, we have developed the FonLiver risk score to predict severe fibrosis, that can be easily incorporated into the routine assessment of patients with Fontan circulation.
Fontan类手术是针对单心室生理的先天性心脏病的一种姑息性手术，从长远来看，可能会导致晚期慢性肝病。在此，我们评估了在Fontan循环背景下评估肝纤维化的传统非侵入性模型的准确性，并开发了一种使用非侵入性工具的新风险评分。
在五个欧洲中心进行了一项前瞻性、横断面观察性研究，纳入所有连续的Fontan循环成年患者、肝活检和非侵入性检查（如弹性成像、APRI和FIB-4）。主要结局是通过活检确定严重肝纤维化。多变量逻辑回归确定了严重纤维化的非侵入性预测因素，从而开发并内部验证了一种名为FonLiver风险评分的新评分模型。
总共纳入了217例患者（平均[标准差]年龄为27.9[8.9]岁；50.7%为男性）。47.9%（95%CI 41.2%-54.5%）存在严重肝纤维化，且与较低的心功能分级、蛋白丢失性肠病以及心肺和全身血流动力学受损相关。最终的FonLiver风险评分纳入了使用瞬时弹性成像测量的肝脏硬度和血小板计数，并显示出很强的区分度和校准度（AUROC为0.81）。FonLiver风险评分优于传统预测模型（如APRI和FIB-4），在我们的队列中，这些传统模型的表现均较差（所有模型的AUROC<0.70）。
Fontan类手术后，严重肝纤维化在成人中很普遍，使用新的FonLiver风险评分可以有效估计。该评分系统可以很容易地纳入Fontan循环患者的常规评估中。
Fontan类手术用作单心室生理先天性心脏病的姑息性手术，从长远来看可能导致晚期慢性肝病。肝纤维化进展的严重程度已被提议作为Fontan血流动力学衰竭以及心脏移植后较差结局的替代指标。FALD筛查方案的制定将有助于早期发现晚期纤维化，并预期采取干预措施以优化Fontan循环，从而改善结局。在我们的国际系列研究中，我们开发了FonLiver风险评分来预测严重纤维化，该评分可轻松纳入Fontan循环患者的常规评估中。
