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

Lange, Charlotte de

doi:10.1007/s00247-020-04682-5

Cited by 9 publications

(10 citation statements)

References 90 publications

(131 reference statements)

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“…. atrioventricular septum defects with unbalanced ventricles (2)(3)(4). A cavopulmonary shunt is manufactured to connect the systemic venous return to the pulmonary arteries without the interposition of an adequate ventricle.…”

Section: Introductionmentioning

confidence: 99%

Long-term management of Fontan patients: The importance of a multidisciplinary approach

et al. 2022

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The Fontan operation is a palliative procedure that leads to increased survival of patients with a functional single ventricle (SV). Starting from 1967 when the first operation was performed by Francis Fontan, more and more patients have reached adulthood. Furthermore, it is expected that in the next 20 years, the population with Fontan circulation will reach 150,000 subjects. The absence of right ventricular propulsion and the inability to improve cardiac output because of the low cardiac reserve are the main issues with the Fontan circulation; however, potential complications may also involve multiple organ systems, such as the liver, lungs, brain, bones, and the lymphatic system. As these patients were initially managed mainly by pediatric cardiologists, it was important to assure the appropriate transition to adult care with the involvement of a multidisciplinary team, including adult congenital cardiologists and multiple subspecialists, many of whom are neither yet familiar with the pathophysiology nor the end-organ consequences of the Fontan circulation. Therefore, the aim of our work was to collect all the best available evidence on Fontan's complications management to provide “simple and immediate” information sources for practitioners looking for state of the art evidence to guide their decision-making and work practices. Moreover, we suggest a model of follow-up of patients with Fontan based on a patient-centered multidisciplinary approach.

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Section: Introductionmentioning

confidence: 99%

Long-term management of Fontan patients: The importance of a multidisciplinary approach

et al. 2022

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“…In addition, the pre-, peri- and post-surgical abnormal hemodynamic condition is likely to contribute to the complications [ 7 ]. These can affect several organs [ 8 ], though the focus of this paper is on Fontan-associated liver disease.…”

Section: Introductionmentioning

confidence: 99%

Surveillance of Fontan-associated liver disease: current standards and a proposal from the European Society of Paediatric Radiology Abdominal Task Force

et al. 2021

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Since Francis Fontan first introduced the eponymous technique, the Fontan procedure, this type of surgical palliation has allowed thousands of children affected by specific heart malformations to reach adulthood. Nevertheless, abdominal, thoracic, lymphatic and neurologic complications are the price that is paid by these patients. Our review focuses on Fontan-associated liver disease; the purpose is to summarize the current understanding of its physiopathology, the aim of follow-up and the specific radiologic follow-up performed in Europe. Finally, we as members of the Abdominal Task Force of the European Society of Paediatric Radiology propose a consensus-based imaging follow-up algorithm.

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“…Complications are, unfortunately, multiple because of the modified circulation as well as the damage occurring in the prenatal and preprocedural period. These complications appear in most pediatric Fontan patients [ 2 ]. Liver complications have recently gained more attention and include fibrosis, often leading to cirrhosis.…”

Section: Introductionmentioning

confidence: 99%

Radiologic follow-up in Fontan-associated liver disease in Europe: European Society of Paediatric Radiology survey demonstrates the need for a consensus protocol

et al. 2021

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Fontan surgery is a life-saving procedure for newborns with complex cardiac malformations, but it originates complications in different organs. The liver is also affected, with development of fibrosis and sometimes cirrhosis and hepatocellular carcinoma. There is no general agreement on how to follow-up these children for the development of liver disease. To understand the current practice on liver follow-up, we invited members of the European Society of Paediatric Radiology (ESPR) to fill out an online questionnaire. The survey comprised seven questions about when and how liver follow-up is performed on Fontan patients. While we found some agreement on the use of US as screening tool, and of MRI for nodule characterization, the discrepancies on timing and the lack of a shared protocol make it currently impossible to compare data among centers.

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

Cited by 9 publications

References 90 publications

Long-term management of Fontan patients: The importance of a multidisciplinary approach

Long-term management of Fontan patients: The importance of a multidisciplinary approach

Surveillance of Fontan-associated liver disease: current standards and a proposal from the European Society of Paediatric Radiology Abdominal Task Force

Radiologic follow-up in Fontan-associated liver disease in Europe: European Society of Paediatric Radiology survey demonstrates the need for a consensus protocol

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