Thrane, Karl Julius;
Müller, Lil Sofie Ording;
Suther, Kathrine Rydén;
Thomassen, Kristian Stien;
Holmström, Henrik;
Thaulow, Erik;
Almaas, Runar;
Möller, Thomas;
de Lange, Charlotte
Spectrum of Fontan-associated liver disease assessed by MRI and US in young adolescents
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Media type:
E-Article
Title:
Spectrum of Fontan-associated liver disease assessed by MRI and US in young adolescents
Contributor:
Thrane, Karl Julius;
Müller, Lil Sofie Ording;
Suther, Kathrine Rydén;
Thomassen, Kristian Stien;
Holmström, Henrik;
Thaulow, Erik;
Almaas, Runar;
Möller, Thomas;
de Lange, Charlotte
imprint:
Springer Science and Business Media LLC, 2021
Description:
<jats:title>Abstract</jats:title><jats:sec>
<jats:title>Purpose</jats:title>
<jats:p>Patients with Fontan circulation are at risk of developing hepatic fibrosis/cirrhosis. The mechanisms and disease development are unclear and early secondary liver cancer is a concern. This study will describe hepatic imaging findings in a national cohort of adolescents with Fontan circulation.</jats:p>
</jats:sec><jats:sec>
<jats:title>Methods</jats:title>
<jats:p>The patients prospectively underwent abdominal contrast enhanced magnetic resonance imaging (MRI) including diffusion-weighted imaging. Images were assessed for criteria of fibrosis<jats:italic>/</jats:italic>cirrhosis including characterization of hepatic nodules. These nodules were in addition, assessed by ultrasonography (US). Nodules ≥ 1 cm were investigated and monitored to evaluate malignant transformation. Clinical and hepatic serological data were recorded.</jats:p>
</jats:sec><jats:sec>
<jats:title>Results</jats:title>
<jats:p>Forty-six patients, median age of 16.5 years (15.4–17.9 years) were enrolled. All patients underwent US examination and MRI was performed in 35/46 patients. On MRI, 60% had hepatomegaly and 37% had signs of fibrosis/cirrhosis. Seven patients had together 13 nodules ≥ 1 cm in diameter. Only 4/13 (17%) where seen on US. Nodules had variable MRI signal characteristics including hepatobiliary contrast enhancement and two nodules revealed portal venous phase ‘wash-out’ on the first examination. No further imaging signs of malignancy were revealed during the follow-up period of median 24.4 (7–42) months.</jats:p>
</jats:sec><jats:sec>
<jats:title>Conclusion</jats:title>
<jats:p>The majority of adolescents with Fontan circulation had imaging findings of fibrosis/cirrhosis of varying severity. US had low detection rate of hepatic nodules compared to MRI. The imaging work-up before transition to adult cardiology care did not reveal findings suggestive of malignancy. However, the high prevalence of Fontan-associated liver disease calls for surveillance strategies even in childhood.</jats:p>
</jats:sec>