Department of Pathology, National Cerebral and Cardiovascular Center, Suita, 5658565, Japan; Department of Legal Medicine, Showa University School of Medicine, Tokyo, 1428555, Japan.
Division of Pediatric Cardiology and Adult Congenital Heart Disease, Department of Perinatal and Pediatric Cardiology, National Cerebral and Cardiovascular Center, Suita, 5658565, Japan.
Pathol Res Pract. 2019 Jun;215(6):152366. doi: 10.1016/j.prp.2019.02.016. Epub 2019 Feb 27.
The Fontan procedure is an open heart procedure performed in pediatric patients with a particular congenital cardiac anomaly known as a univentricular heart. The procedure is used to reroute the systemic venous blood from the inferior vena cava directly to the pulmonary artery. It improves patients' prognoses, but various late-phase extracardiac complications that manifest when patients reach adolescence have been recognized. These complications, pulmonary arteriovenous fistula and protein losing gastroenteropathy, for example, present significant challenges in the management of adults with Fontan circulation. Liver fibrosis is another possible late-phase complication and one of the most serious. Development of a neoplasm, usually a hepatocellular carcinoma, is sometimes reported. We encountered a young patient in whom Fontan circulation led to the development of a histologically unusual liver cancer that resembled the poorly differentiated hepatocellular carcinoma or the combined hepatocellular-cholangiocarcinoma with stem-cell features described in the latest WHO classification.
法乐四联症根治术是一种在儿科患者中进行的心脏直视手术，这些患者患有一种特定的先天性心脏畸形，称为单心室心脏。该手术用于将体循环静脉血从下腔静脉直接重新引导至肺动脉。它改善了患者的预后，但当患者进入青春期时，已经认识到各种晚期的心脏外并发症。这些并发症，例如肺动静脉瘘和蛋白丢失性胃肠病，在成人法乐四联症患者的管理中带来了重大挑战。肝纤维化是另一种可能的晚期并发症，也是最严重的并发症之一。发展为肿瘤，通常是肝细胞癌，有时也有报道。我们遇到了一名年轻患者，法乐四联症导致其发展为一种组织学上不常见的肝癌，类似于最新的世界卫生组织分类中描述的低分化肝细胞癌或具有干细胞特征的肝细胞-胆管细胞癌。
