Abdominal imaging findings in adult patients with Fontan circulation
Tae-Hyung Kim1,2, Hyun Kyung Yang3, Hyun-Jung Jang3
1Department of Radiology, Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul, 03080, South Korea.
Insights
The Fontan procedure improves survival for single-ventricle patients into adulthood. Radiologists must recognize Fontan-associated liver disease and other abdominal issues like nodules, tumors, and enteropathy on imaging.
Area of Science:
- Cardiology
- Radiology
- Hepatology
Background:
- The Fontan procedure enhances survival for pediatric patients with functional single ventricles into adulthood.
- Fontan circuit physiology elevates systemic venous pressure, potentially causing long-term multi-system organ failure.
- Fontan-associated liver disease (FALD) is a significant complication, progressing to cirrhosis and portal hypertension.
Purpose of the Study:
- To highlight common abdominal abnormalities in post-Fontan patients.
- To familiarize radiologists with imaging findings in Fontan-associated liver disease (FALD).
- To emphasize the importance of recognizing conditions like focal nodular hyperplasia-like nodules, hepatocellular carcinoma, protein-losing enteropathy, and pheochromocytoma/paraganglioma.
Main Methods:
- Review of imaging findings in post-Fontan patients.
- Correlation of imaging findings with clinical outcomes.
- Emphasis on cross-sectional imaging modalities.
Main Results:
- Focal nodular hyperplasia-like nodules are common in FALD.
- Hepatocellular carcinoma infrequently develops in FALD.
- Other potential abdominal abnormalities include protein-losing enteropathy and pheochromocytoma/paraganglioma.
Conclusions:
- The Fontan procedure has improved long-term survival.
- Radiologists need to be aware of unique imaging features of FALD.
- Early recognition of associated abdominal conditions is crucial for patient management.
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.
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