Department of Radiology, Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave., Cincinnati, OH, 45229, USA.
Department of Radiology, University of Cincinnati College of Medicine, Cincinnati, OH, USA.
Pediatr Radiol. 2020 Oct;50(11):1528-1541. doi: 10.1007/s00247-020-04776-0. Epub 2020 Aug 18.
The Fontan operation has dramatically altered the natural history of functionally single ventricle congenital heart disease. Patients who have undergone the Fontan operation are living longer and, thus, noncardiac morbidity resulting from the Fontan operation is increasingly being recognized. Fontan-associated liver disease (FALD), one of the chief morbidities following the Fontan operation, is believed to be a multifactorial process that manifests as hepatic congestion and fibrosis, portal hypertension, and development of focal liver lesions, including malignant tumors. This article reviews the imaging findings of FALD in the pediatric and young adult population, reviews the literature related to the imaging of FALD and discusses possible screening algorithms for this population. The need for further research to better understand the causes of FALD, to establish if early liver stiffness measurements (or their change over time) predict long-term outcomes and complications, and to define optimal liver screening procedures is highlighted.
法洛四联症根治术后显著改变了功能性单心室先天性心脏病的自然史。接受过法洛四联症根治术的患者寿命更长，因此，法洛四联症根治术后非心脏并发症日益受到重视。法洛四联症相关肝疾病（FALD）是法洛四联症根治术后的主要并发症之一，据信是一种多因素的过程，表现为肝淤血和纤维化、门静脉高压以及局灶性肝病变的发展，包括恶性肿瘤。本文综述了儿科和青年人群中法洛四联症相关肝疾病的影像学表现，回顾了与 FALD 影像学相关的文献，并讨论了该人群可能的筛查方案。强调需要进一步研究以更好地了解 FALD 的病因，确定早期肝硬度测量（或其随时间的变化）是否预测长期预后和并发症，并确定最佳的肝脏筛查程序。
