Spectrum of Fontan-associated liver disease assessed by MRI and US in young adolescents.

Autor: Thrane KJ; Div of Radiology and Nuclear Medicine, Section of Paediatric Radiology, Oslo University Hospital, Oslo, Norway., Müller LSO; Div of Radiology and Nuclear Medicine, Section of Paediatric Radiology, Oslo University Hospital, Oslo, Norway., Suther KR; Div of Radiology and Nuclear Medicine, Section of Paediatric Radiology, Oslo University Hospital, Oslo, Norway., Thomassen KS; Div of Radiology and Nuclear Medicine, Section of Paediatric Radiology, Oslo University Hospital, Oslo, Norway., Holmström H; Dept of Paediatric Cardiology, Oslo University Hospital, Oslo, Norway.; Institute of Clinical Medicine, University of Oslo, Oslo, Norway., Thaulow E; Dept of Paediatric Cardiology, Oslo University Hospital, Oslo, Norway.; Institute of Clinical Medicine, University of Oslo, Oslo, Norway., Almaas R; Dept of Paediatric Research and Div of Paediatric and Adolescent Medicine, Oslo University Hospital, Oslo, Norway., Möller T; Dept of Paediatric Cardiology, Oslo University Hospital, Oslo, Norway., de Lange C; Div of Radiology and Nuclear Medicine, Section of Paediatric Radiology, Oslo University Hospital, Oslo, Norway. charlotte.de.lange@vgregion.se.; Dept of Radiology and Clinical Physiology Queen Silvia Childrens' Hospital, Sahlgrenska University Hospital, Göteborg, Sweden. charlotte.de.lange@vgregion.se.
Jazyk: angličtina
Zdroj: Abdominal radiology (New York) [Abdom Radiol (NY)] 2021 Jul; Vol. 46 (7), pp. 3205-3216. Date of Electronic Publication: 2021 Mar 10.
DOI: 10.1007/s00261-021-02994-0
Abstrakt: Purpose: 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.
Methods: The patients prospectively underwent abdominal contrast enhanced magnetic resonance imaging (MRI) including diffusion-weighted imaging. Images were assessed for criteria of fibrosis/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.
Results: 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.
Conclusion: 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.
Databáze: MEDLINE
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