Madala Samragnyi, MacDougall Kira, Polavarapu Abhishek, Gurala Dinesh, Gumaste Vivek, Morvillo Gerard
Department of Internal Medicine, Staten Island University Hospital, New York, New York, USA.
Department of Gastroenterology, Staten Island University Hospital, New York, New York, USA.
Case Rep Gastroenterol. 2020 Nov 23;14(3):615-623. doi: 10.1159/000510332. eCollection 2020 Sep-Dec.
The Fontan procedure is a surgical procedure for patients with single-ventricle anatomy that results in the flow of systemic venous blood to the lungs without passing through a ventricle. Before the 1970s, most children with single-ventricle anatomy failed to survive into adulthood. With the introduction of the Fontan procedure, and its many modifications, the survival rate of these patients improved exponentially. With patients surviving longer, complications from this procedure are being documented for the first time. Cardiovascular complications are expected early on and are well studied. More serious are the non-cardiovascular complications in patients who survive into adulthood. The biggest entity is Fontan-associated liver disease (FALD) which needs thorough monitoring to screen for hepatocellular carcinoma (HCC). FALD includes chronic passive congestion, liver cirrhosis, and HCC. Once cirrhosis develops, monitoring with annual liver function tests, AFP, and abdominal ultrasonography need to occur to screen for HCC. Patients may need to be evaluated for combined heart-liver transplantation. Strict guidelines need to be developed for monitoring and surveillance of these patients to prevent late-stage complications. Herein, we report a unique case of FALD in a young female presenting two decades after the procedure with variceal bleeding.
Fontan手术是针对单心室解剖结构患者的一种外科手术，该手术可使体循环静脉血不经过心室而流入肺部。在20世纪70年代之前，大多数单心室解剖结构的儿童无法存活至成年。随着Fontan手术及其众多改良术式的引入，这些患者的生存率呈指数级提高。随着患者存活时间延长，该手术的并发症首次被记录下来。心血管并发症在早期就可预期且已有充分研究。对于存活至成年的患者，更严重的是非心血管并发症。最主要的是Fontan相关肝病（FALD），需要进行全面监测以筛查肝细胞癌（HCC）。FALD包括慢性被动性充血、肝硬化和HCC。一旦发生肝硬化，需要每年进行肝功能检查、甲胎蛋白检测和腹部超声检查以筛查HCC。患者可能需要接受心肝联合移植评估。需要制定严格的指南来监测和随访这些患者，以预防晚期并发症。在此，我们报告一例FALD的独特病例，该年轻女性在手术后二十年出现静脉曲张出血。
