Abstract

PurposePatients 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.MethodsThe 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.ResultsForty-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.ConclusionThe 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.

Highlights

  • Children born with univentricular heart defects are surgically palliated with the Fontan procedure, where in the United States an average of 1062 operations are performed per year [1]

  • We describe the range of hepatic imaging findings in US and magnetic resonance imaging (MRI), including findings on a longitudinal follow-up of a median of 24.4 months in a subgroup

  • In a national cohort of young adolescents with Fontan palliation, hepatic changes including nodules presented on MRI and US, were highly prevalent

Read more

Summary

Introduction

Children born with univentricular heart defects are surgically palliated with the Fontan procedure, where in the United States an average of 1062 operations are performed per year [1]. Worldwide the estimation is that 70.000 patients are living with Fontan circulation. Refinements of the surgical technique and peri/postoperative care over the last decades have largely improved the prospects of survival into adulthood [2]. Long-term complications of this palliative circulation are recognized, affecting the heart itself but several end organs. The negative impact on the liver is of special and growing concern [3, 4]. In the Fontan circulation, one functional cardiac chamber pumps blood to the systemic side, while the systemic

Objectives
Methods
Results
Discussion
Conclusion
Full Text
Published version (Free)

Talk to us

Join us for a 30 min session where you can share your feedback and ask us any queries you have

Schedule a call