Imaging of Fontan-associated liver disease
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REVIEW
Imaging of Fontan-associated liver disease Jonathan R. Dillman 1,2
&
Andrew T. Trout 1,2,3 & Tarek Alsaied 3,4 & Anita Gupta 5 & Adam M. Lubert 3,4
Received: 29 March 2020 / Revised: 1 June 2020 / Accepted: 6 July 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract 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. Keywords Children . Congestive hepatopathy . Fontan-associated liver disease . Fontan circulation . Liver . Magnetic resonance imaging . Single ventricle congenital heart disease . Ultrasound . Young adults
Introduction Congenital heart diseases with single ventricle physiology are malformations that functionally result in a single ventricle, as CME activity This article has been selected as the CME activity for the current month. Please visit the SPR website at www.pedrad.org on the Education page and follow the instructions to complete this CME activity. * Jonathan R. Dillman [email protected] 1
Department of Radiology, Cincinnati Children’s Hospital Medical Center, 3333 Burnet Ave., Cincinnati, OH 45229, USA
2
Department of Radiology, University of Cincinnati College of Medicine, Cincinnati, OH, USA
3
Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA
4
Heart Institute, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA
5
Department of Pathology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA
either the right or left ventricle is underdeveloped [1]. Surgical palliation involves several successive stages, culminating in the Fontan operation [2]. The Fontan operation, originally described by the French cardiothoracic surgeon Francis Fontan as a treatment for tricuspid atresia, was first performed in 1968 and reported in 1971 as a method to surgically palliate such a cardiac lesion [3]. The early Fontan operations were performed as an anastomosis between the right atrium and pulmonary artery (atriopulmonary Fontan). However, to avoid the hemodynamic challenges of the atriopulmonary Fontan, th
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