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Vol. 63. Issue 2.
Pages 159-169 (March - April 2021)
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Vol. 63. Issue 2.
Pages 159-169 (March - April 2021)
Update in Radiology
Fontan-associated liver disease
Enfermedad hepática crónica asociada a la cirugía de Fontan
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M.J. Parada Blázqueza,
Corresponding author
joseparadab86@gmail.com

Corresponding author.
, D. Rodríguez Vargasb, J. Mohigefer Barrerac, J.J. Borrero Martínc, B. Vargas Serranoa
a Unidad de Gestión Clínica de Radiodiagnóstico, Hospital Universitario Virgen del Rocío, Sevilla, Spain
b Unidad de Gestión Clínica de Radiodiagnóstico, Hospital Juan Ramón Jiménez, Huelva, Spain
c Unidad de Gestión Clínica de Anatomía Patológica, Hospital Universitario Virgen del Rocío, Sevilla, Spain
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Table 1. Summary of radiological characteristics of the parenchyma and the nodules in Fontan-associated chronic liver disease.
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:
Fontan surgery
Fontan-associated liver disease
Hepatic fibrosis
Focal nodular hyperplasia (FNH)-like
Hepatocellular carcinoma
Resumen
Objetivo

Revisar la fisiopatología de la enfermedad hepática crónica asociada a la cirugía de Fontan, sus cambios histológicos y sus manifestaciones radiológicas.

Conclusiones

La enfermedad hepática crónica asociada a la cirugía de Fontan corresponde al conjunto de cambios estructurales y funcionales hepáticos secundarios a las modificaciones hemodinámicas propias de esta cirugía. Sus manifestaciones radiológicas comprenden: alteraciones en el tamaño y contorno hepático, alteraciones en el patrón de realce o intensidad de señal, anomalías en las estructuras vasculares y lesiones focales, que incluyen nódulos benignos hipercaptantes en fase arterial y carcinoma hepatocelular. El radiólogo debe de estar familiarizado con esta patología y sus complicaciones, puesto que los pacientes con cirugía de Fontan representan una población cada vez mayor, candidata a un número creciente de pruebas de imagen.

Palabras clave:
Cirugía de Fontan
Enfermedad hepática crónica asociada al Fontan
Fibrosis hepática
Hiperplasia nodular focal-like
Carcinoma hepatocelular

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