Tsuchihashi Tomoya, Cho Yuki, Tokuhara Daisuke
Department of Pediatrics, Wakayama Medical University, Wakayama, Japan.
Department of Pediatrics, Osaka Metropolitan University, Osaka, Japan.
Front Med (Lausanne). 2024 Nov 27;11:1354857. doi: 10.3389/fmed.2024.1354857. eCollection 2024.
The Fontan operation, which directly connects the superior and inferior vena cava to the pulmonary artery, is a palliative surgery for children with a functional or anatomic single ventricle. This procedure leads to hemodynamic changes (Fontan circulation) in patients, who tend to develop congestive hepatic fibrosis characterized by sinusoidal fibrosis and dilatation beginning approximately 10 years after the procedure. In addition, in the context of severe fibrosis and cirrhosis, hepato-gastrointestinal complications including hepatocellular carcinoma, focal nodular hyperplasia, and portal hypertension can arise. Fontan-associated liver disease (FALD) encompasses the broad spectrum of liver alterations secondary to postoperative hemodynamic changes, and the effective management of FALD requires contributions from specialists in hepatology, gastroenterology, surgery, radiology, histopathology, and pediatric and adult cardiology. In this article, we outline the pathogenesis of FALD and discuss the importance of a multidisciplinary collaborative approach to its management.
Fontan手术直接将上、下腔静脉与肺动脉相连，是针对功能性或解剖性单心室患儿的一种姑息性手术。该手术会导致患者出现血流动力学变化（Fontan循环），患者大约在术后10年往往会发展为以窦性纤维化和扩张为特征的充血性肝纤维化。此外，在严重纤维化和肝硬化的情况下，可能会出现包括肝细胞癌、局灶性结节性增生和门静脉高压在内的肝胃肠并发症。Fontan相关肝病（FALD）涵盖了术后血流动力学变化继发的广泛肝脏改变，FALD的有效管理需要肝病学、胃肠病学、外科、放射学、组织病理学以及儿科和成人心脏病学专家的共同努力。在本文中，我们概述了FALD的发病机制，并讨论了多学科协作方法对其管理的重要性。
