Fontan-associated liver disease

• Published in: Radiologia Vol. 63; no. 2; pp. 159 - 169
• Main Authors: Parada Blázquez, M J, Rodríguez Vargas, D, Mohigefer Barrera, J, Borrero Martín, J J, Vargas Serrano, B
• Format: Journal Article
• Language: English; Spanish
• Published: Spain 01.03.2021
• Subjects: Hepatic fibrosis; Carcinoma hepatocelular; Hiperplasia nodular focal-like; Cirugía de Fontan; Fibrosis hepática; Enfermedad hepática crónica asociada al Fontan; Fontan surgery; Hepatocellular carcinoma; Focal nodular hyperplasia (FNH)-like; Fontan-associated liver disease
• ISSN: 1578-178X; 2173-5107
• DOI: 10.1016/j.rx.2020.10.010

To review the pathophysiology of Fontan-associated liver disease, its histologic changes, and its radiologic manifestations. Fontan-associated liver disease is the result of a set of structural and functional changes in the liver that occur secondary to hemodynamic changes brought about by Fontan surgery. The radiologic manifestations of Fontan-associated liver disease consist of changes in the size and shape of the liver, alterations in the signal intensity or pattern of enhancement, abnormalities in the vascular structures, and focal lesions, which include benign nodules with intense uptake in the arterial phase and hepatocellular carcinoma. Radiologists need to be familiar with this disease and its complications, because the number of patients who undergo Fontan surgery continues to increase, and these patients undergo an increasing number of imaging tests.