Babaoglu Kadir, Binnetoglu Fatih Köksal, Aydoğan Ayşen, Altun Gürkan, Gürbüz Yeşim, Inan Nagihan, Corapçioğlu Funda
Department of Pediatric Cardiology, Faculty of Medicine, Kocaeli University, Kocaeli, Turkey.
Pediatr Cardiol. 2010 Aug;31(6):861-4. doi: 10.1007/s00246-010-9685-x. Epub 2010 Mar 4.
Patients who undergo a Fontan procedure experience some degree of liver disease. Hemodynamic changes such as central venous hypertension, depressed dynamic cardiac output, and late ventricular dysfunction combined with long-standing hypoxia preceding the Fontan procedure all are recognized risk factors for hepatic injury. The histopathologic changes associated with cardiac hepatopathy include chronic passive congestion, centrilobular necrosis, and cardiac cirrhosis. However, hepatic adenoma and hepatic adenomatosis (HA) are not well-known pathologies during the course of cardiac hepatopathy. This study focused on a 7-year-old girl with chronic hepatic changes and HA who had undergone a Fontan procedure. Hepatic adenomatosis was diagnosed on the basis of magnetic resonance imaging (MRI) and MRI-guided liver biopsy. To the best of the authors' knowledge, this case involved the youngest patient with hepatocellular adenomatosis documented in the literature. It was a unique case because the patient experienced HA after a Fontan procedure.
接受Fontan手术的患者会出现一定程度的肝脏疾病。诸如中心静脉高压、动态心输出量降低以及晚期心室功能障碍等血流动力学变化，再加上Fontan手术前长期存在的缺氧，这些都是公认的肝损伤危险因素。与心性肝病相关的组织病理学变化包括慢性被动性充血、小叶中心坏死和心源性肝硬化。然而，肝腺瘤和肝腺瘤病（HA）在心性肝病过程中并非广为人知的病变。本研究聚焦于一名接受过Fontan手术、患有慢性肝脏改变和肝腺瘤病的7岁女孩。肝腺瘤病是根据磁共振成像（MRI）和MRI引导下的肝活检确诊的。据作者所知，该病例是文献记载中最年轻的肝细胞腺瘤病患者。这是一个独特的病例，因为该患者在Fontan手术后出现了肝腺瘤病。
