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Insights Imaging. 2018 Jun;9(3):357-367. doi: 10.1007/s13244-018-0609-2. Epub 2018 Apr 5.

Abdominal imaging findings in adult patients with Fontan circulation.

Author information

1
Department of Radiology, Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul, 03080, South Korea.
2
Department of Radiology, Seoul National University College of Medicine, 103 Daehak-ro, Jongnogu, Seoul, 03080, South Korea.
3
Department of Medical Imaging, Toronto General Hospital, 585 University Avenue, Toronto, ON, M5G 2N2, Canada.
4
Department of Diagnostic Imaging, Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5J2L4, Canada.
5
Department of Medical Imaging, Toronto General Hospital, 585 University Avenue, Toronto, ON, M5G 2N2, Canada. taekyoung.kim@uhn.ca.

Abstract

The Fontan procedures, designed to treat paediatric patients with functional single ventricles, have markedly improved the patient's survival into adulthood. The physiology of the Fontan circuit inevitably increases systemic venous pressure, which may lead to multi-system organ failure in the long-term follow-up. Fontan-associated liver disease (FALD) can progress to liver cirrhosis with signs of portal hypertension. Focal nodular hyperplasia-like nodules commonly develop in FALD. Imaging surveillance is often performed to monitor the progression of FALD and to detect hepatocellular carcinoma, which infrequently develops in FALD. Other abdominal abnormalities in post-Fontan patients include protein losing enteropathy and pheochromocytoma/paraganglioma. Given that these abdominal abnormalities are critical for patient management, it is important for radiologists to become familiar with the abdominal abnormalities that are common in post-Fontan patients on cross-sectional imaging.

TEACHING POINTS:

• Fontan procedure for functional single ventricle has improved patient survival into adulthood. • Radiologists should be familiar with unique imaging findings of Fontan-associated liver disease. • Focal nodular hyperplasia-like nodules commonly develop in Fontan-associated liver disease. • Hepatocellular carcinoma, protein-losing enteropathy, pheochromocytoma/paraganglioma may develop.

KEYWORDS:

Diagnostic imaging; Digestive system neoplasms; Focal nodular hyperplasia; Heart defects, congenital; Liver cirrhosis

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