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 Into Imaging
|April 7, 2018
PubMed

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.

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