Department of Diagnostic Imaging, The Hospital For Sick Children, 555 University Ave, Toronto, ON M5G 1X8, Canada.
Medical Imaging Department, University of Toronto, Toronto, ON, Canada.
AJR Am J Roentgenol. 2021 Jul;217(1):207-217. doi: 10.2214/AJR.20.23404. Epub 2021 Apr 28.
The Fontan procedure has significantly improved the survival in children with a functional single ventricle, but it is associated with chronically elevated systemic venous pressure that leads to multisystemic complications. Imaging plays an important role in assessing these complications and guiding management. The pathophysiology, imaging modalities, and current surveillance recommendations are discussed and illustrated. Significant improvement in survival of patients with Fontan circulation is associated with ongoing cardiac and extracardiac comorbidities and multisystemic complications. The liver and intestines are particularly vulnerable to damage. In addition, this patient population has been shown to be at increased risk of certain malignancies such as hepatocellular carcinoma and neuroendocrine tumors. Familiarity with imaging findings of Fontan-associated liver disease and other abdominal complications of the Fontan circulation is essential for radiologists because we are likely to encounter these patients in our general practice.
法洛四联症根治术后患儿的生存率显著提高，但与慢性全身性静脉高压相关，进而导致多系统并发症。影像学在评估这些并发症和指导治疗方面发挥着重要作用。本文讨论并图示了法洛四联症根治术后的病理生理学、影像学方法和当前监测建议。法洛四联症根治术后患者的生存率显著提高，同时伴有持续性的心脏和心脏外合并症及多系统并发症。肝脏和肠道特别容易受到损害。此外，该患者人群已被证实存在某些恶性肿瘤（如肝细胞癌和神经内分泌肿瘤）的风险增加。放射科医生需要熟悉法洛四联症根治术后肝脏疾病和法洛四联症根治术后循环的其他腹部并发症的影像学表现，因为我们在日常工作中可能会遇到这些患者。
