Fontan-associated liver disease

M J Parada Blázquez; D Rodríguez Vargas; J Mohigefer Barrera; J J Borrero Martín; B Vargas Serrano

doi:10.1016/j.rx.2020.10.010

Fontan-associated liver disease

Radiologia (Engl Ed). 2021 Mar-Apr;63(2):159-169. doi: 10.1016/j.rx.2020.10.010. Epub 2021 Jan 13.

[Article in English, Spanish]

Authors

M J Parada Blázquez¹, D Rodríguez Vargas², J Mohigefer Barrera³, J J Borrero Martín³, B Vargas Serrano⁴

Affiliations

¹ Unidad de Gestión Clínica de Radiodiagnóstico, Hospital Universitario Virgen del Rocío, Sevilla, España. Electronic address: joseparadab86@gmail.com.
² Unidad de Gestión Clínica de Radiodiagnóstico, Hospital Juan Ramón Jiménez, Huelva, España.
³ Unidad de Gestión Clínica de Anatomía Patológica, Hospital Universitario Virgen del Rocío, Sevilla, España.
⁴ Unidad de Gestión Clínica de Radiodiagnóstico, Hospital Universitario Virgen del Rocío, Sevilla, España.

PMID: 33451720
DOI: 10.1016/j.rx.2020.10.010

Abstract

Objective: To review the pathophysiology of Fontan-associated liver disease, its histologic changes, and its radiologic manifestations.

Conclusions: 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.

Keywords: Carcinoma hepatocelular; Cirugía de Fontan; Enfermedad hepática crónica asociada al Fontan; Fibrosis hepática; Focal nodular hyperplasia (FNH)-like; Fontan surgery; Fontan-associated liver disease; Hepatic fibrosis; Hepatocellular carcinoma; Hiperplasia nodular focal-like.