Armstrong Paul, Moriarty Aoife, Hughes Robert, Mehigan Niamh, Savage Rhona, Walsh Kevin, Russell Jennifer, Stewart Stephen
The Liver Centre, Mater Misericordiae University Hospital, Dublin, Ireland
School of Medicine, University College Dublin, Dublin, Ireland.
BMJ Open Gastroenterol. 2025 Aug 5;12(1):e001733. doi: 10.1136/bmjgast-2024-001733.
OBJECTIVE: Fontan-associated liver disease (FALD) results from haemodynamic changes following the Fontan procedure for congenital heart disease and is associated with poorer outcomes. The prevalence of Fontan is rising due to improved survival; however, little is known about predictors of advanced liver fibrosis in adult patients. This study aimed to determine the accuracy of non-invasive fibrosis assessment tools (NIT) in predicting histologically confirmed advanced liver fibrosis in an adult Fontan cohort attending Mater Misericordiae University Hospital. METHODS: Patient demographics, congenital cardiac variables and fibrosis biomarkers were recorded including liver stiffness measurement (LSM) via transient elastography, Fibrosis-4 (FIB-4) and Aspartate aminotransferase-to-Platelet Ratio Index (APRI) scores. Biopsies, taken between 2017 and 2024, were staged using the congestive hepatic fibrosis score. Analysis was performed using SPSS. RESULTS: 71 patients (58% male) were included. The median age was 25 years. 62% had histological advanced fibrosis. There were no significant bleeding events post biopsy. Overall, advanced fibrosis was associated with a closed Fontan fenestration (p=0.022) and higher LSM, although with a weak correlation (p=0.04, r=0.25, area under the curve (AUC) 0.65), but not with APRI or FIB-4. There was no difference in rates of advanced fibrosis between sex (p=0.84). In females, higher APRI was associated with advanced fibrosis (p=0.045, r=0.41, AUC 0.73). CONCLUSIONS: The majority of Fontan patients have advanced liver fibrosis in their third decade. A patent Fontan fenestration appears to reduce the risk of advanced fibrosis. Despite an association with higher LSM, there was no cut-off which could negate the need for biopsy in a significant population. Our data suggest that the discriminatory ability of NIT may vary according to sex. Liver biopsy is safe and remains the only method of reliably diagnosing advanced fibrosis in FALD.
目的:Fontan相关肝病(FALD)是先天性心脏病Fontan手术后继发性血流动力学改变所致,与较差的预后相关。由于生存率提高,Fontan手术的患病率正在上升;然而,对于成年患者晚期肝纤维化的预测因素知之甚少。本研究旨在确定非侵入性纤维化评估工具(NIT)在预测圣母玛利亚大学医院成年Fontan队列中经组织学证实的晚期肝纤维化方面的准确性。 方法:记录患者的人口统计学资料、先天性心脏变量和纤维化生物标志物,包括通过瞬时弹性成像测量肝脏硬度(LSM)、Fibrosis-4(FIB-4)和天冬氨酸转氨酶与血小板比值指数(APRI)评分。2017年至2024年间采集的活检标本采用充血性肝纤维化评分进行分期。使用SPSS进行分析。 结果:纳入71例患者(58%为男性)。中位年龄为25岁。62%有组织学上的晚期纤维化。活检后无明显出血事件。总体而言,晚期纤维化与Fontan开窗关闭有关(p=0.022),与较高的LSM有关,尽管相关性较弱(p=0.04,r=0.25,曲线下面积(AUC)0.65),但与APRI或FIB-4无关。性别间晚期纤维化发生率无差异(p=0.84)。在女性中,较高的APRI与晚期纤维化有关(p=0.045,r=0.41,AUC 0.73)。 结论:大多数Fontan患者在第三个十年就出现了晚期肝纤维化。Fontan开窗未闭似乎可降低晚期纤维化的风险。尽管与较高的LSM有关,但没有一个临界值可以使大量人群无需活检。我们的数据表明,NIT的鉴别能力可能因性别而异。肝活检是安全的,仍然是可靠诊断FALD中晚期纤维化的唯一方法。
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