Imaging of complications following Fontan circulation in children — diagnosis and surveillance

The Fontan operation is performed for various cardiac lesions with single-ventricle physiology. The survival rate of Fontan patients is increasing for adolescents and young adults, with an expected 30-year survival of >80%. Medical health care providers including specialists in organ systems and pediatric radiologists need to improve their knowledge about the Fontan circulation and the various organ complications to monitor care. In this review the author explains the basic anatomical and functional features of Fontan palliation and gives an overview of the multiple long-term organ complications that might present in the pediatric population. These include decreased physical capacity, ventricular dysfunction, atrioventricular valve regurgitation and arrhythmia, as well as protein-losing enteropathy, plastic bronchitis, growth/bone composition disturbances, renal dysfunction, and the recently recognized and important liver fibrosis (Fontan-associated liver disease). Neuropsychological and behavioral deficits occur frequently. This review focuses on the key role of radiology in making the diagnosis of these complications, monitoring therapy and predicting outcomes in the pediatric age group. The author discusses how and when radiology is important in Fontan patients, as well as how new techniques enabling quantitative measures in imaging with US, MRI and CT are adapted for pediatric use, and how they contribute to urgently needed surveillance strategies. ELECTRONIC SUPPLEMENTARY MATERIAL: The online version of this article (10.1007/s00247-020-04682-5) contains supplementary material, which is available to authorized users.