Imaging of Fontan-associated liver disease. 2020

Jonathan R Dillman, and Andrew T Trout, and Tarek Alsaied, and Anita Gupta, and Adam M Lubert
Department of Radiology, Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave., Cincinnati, OH, 45229, USA. jonathan.dillman@cchmc.org.

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.

UI MeSH Term Description Entries
D008107 Liver Diseases Pathological processes of the LIVER. Liver Dysfunction,Disease, Liver,Diseases, Liver,Dysfunction, Liver,Dysfunctions, Liver,Liver Disease,Liver Dysfunctions
D011183 Postoperative Complications Pathologic processes that affect patients after a surgical procedure. They may or may not be related to the disease for which the surgery was done, and they may or may not be direct results of the surgery. Complication, Postoperative,Complications, Postoperative,Postoperative Complication
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D006330 Heart Defects, Congenital Developmental abnormalities involving structures of the heart. These defects are present at birth but may be discovered later in life. Congenital Heart Disease,Heart Abnormalities,Abnormality, Heart,Congenital Heart Defect,Congenital Heart Defects,Defects, Congenital Heart,Heart Defect, Congenital,Heart, Malformation Of,Congenital Heart Diseases,Defect, Congenital Heart,Disease, Congenital Heart,Heart Abnormality,Heart Disease, Congenital,Malformation Of Heart,Malformation Of Hearts
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D055815 Young Adult A person between 19 and 24 years of age. Adult, Young,Adults, Young,Young Adults
D018729 Fontan Procedure A procedure in which total right atrial or total caval blood flow is channeled directly into the pulmonary artery or into a small right ventricle that serves only as a conduit. The principal congenital malformations for which this operation is useful are TRICUSPID ATRESIA and single ventricle with pulmonary stenosis. Fontan Circuit,Fontan Circulation,Fontan Operation,Fontan Palliation,Hemi-Fontan Procedure,Bidirectional Cavopulmonary Shunt,Bidirectional Glenn Procedure,Bidirectional Glenn Shunt,Norwood Procedure, Stage 2,Norwood Procedure, Stage 3,Norwood Procedure, Stage II,Norwood Procedure, Stage III,Stage 2 Norwood Procedure,Stage 3 Norwood Procedure,Stage II Norwood Procedure,Stage III Norwood Procedure,Bidirectional Cavopulmonary Shunts,Bidirectional Glenn Procedures,Bidirectional Glenn Shunts,Cavopulmonary Shunt, Bidirectional,Circuit, Fontan,Circulation, Fontan,Glenn Procedure, Bidirectional,Glenn Shunt, Bidirectional,Hemi Fontan Procedure,Operation, Fontan,Palliation, Fontan,Procedure, Bidirectional Glenn,Procedure, Fontan,Procedure, Hemi-Fontan,Shunt, Bidirectional Cavopulmonary

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