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Magnetic resonance imaging identifies unsuspected liver abnormalities in patients after the Fontan procedure
Ozlem Pinar Bulut1, Rene Romero, William T Mahle
1Division of Pediatric Gastroenterology, Emory University School of Medicine, Children's Healthcare of Atlanta, Atlanta, GA 30322, USA.
Insights
Abdominal magnetic resonance imaging (MRI) detects liver changes in Fontan procedure patients before clinical signs appear. This imaging can reveal hepatic abnormalities missed by standard tests, aiding early detection in Fontan survivors.
Area of Science:
- Cardiology
- Hepatology
- Radiology
Background:
- The Fontan procedure is a palliative surgery for complex congenital heart defects.
- Patients undergoing the Fontan procedure are at risk for hepatic complications.
- Early detection of liver abnormalities is crucial for managing Fontan-associated liver disease.
Purpose of the Study:
- To evaluate the utility of abdominal magnetic resonance imaging (MRI) in detecting subclinical hepatic abnormalities in patients post-Fontan procedure.
- To compare MRI findings with clinical and biochemical assessments in this patient cohort.
Main Methods:
- Retrospective review of 39 children and adolescents who underwent the Fontan procedure.
- Analysis of clinical examination findings, liver function tests, and abdominal MRI results.
- Blinded evaluation of MRI scans by two radiologists to assess liver morphology and enhancement patterns.
Main Results:
- All patients had normal liver function tests and no evidence of chronic liver disease.
- Abdominal MRI revealed morphologic liver changes, including reticular contrast enhancement consistent with fibrosis and congestion, in all scanned patients (67% of the cohort).
- Non-uniform enhancement and multifocal arterially enhancing lesions were observed in a subset of patients.
Conclusions:
- Abdominal MRI is a sensitive tool for identifying hepatic abnormalities in Fontan procedure patients, often preceding clinical or biochemical manifestations.
- The non-uniform distribution of abnormalities suggests potential limitations of liver biopsy in this population.
- MRI findings highlight the need for vigilant hepatic monitoring in Fontan survivors.
Objective:
To determine whether abdominal magnetic resonance imaging (MRI) detects hepatic abnormalities before clinical or biochemical perturbations in patients after the Fontan procedure.
Study Design:
Thirty-nine children and adolescents who underwent the Fontan procedure and were referred to a pediatric hepatologist by cardiology services between 2011 and 2012 were reviewed retrospectively. Physical examination findings, routine laboratory tests of liver function, evaluation for chronic liver disease, and abdominal MRI findings were recorded. MRI findings were evaluated relative to time elapsed since surgery by 2 radiologists (blinded).
Results:
Assessment for coexisting chronic liver disease was negative in all patients. All patients had a normal serum albumin level and International Normalized Ratio. Twenty-six of the 39 patients (67%) underwent abdominal MRI, 4 had MRI-incompatible hardware, and 9 did not undergo MRI because of insurance denial. All MRI scans demonstrated morphologic liver changes with varying degrees of reticular contrast enhancement compatible with fibrosis and congestion. Reticular contrast enhancement was often nonuniform, and 9 patients (35%) had multifocal arterially enhancing lesions.
Conclusion:
MRI can identify hepatic abnormalities in patients after Fontan surgery that go undetected by standard clinical and laboratory assessments. These abnormalities are not uniformly distributed throughout the liver, and thus assessment by liver biopsy analysis is subject to sampling error.
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