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Research Article: MRI-derived IVIM-modelled diffusion-weighted imaging does not predict clinical outcomes in fontan-associated liver disease: to monitor or not to monitor the liver?

Date Published: 2026-06-22

Abstract:
Fontan-associated liver disease (FALD) encompasses the spectrum of hepatic alterations in patients after Fontan palliation. FALD can progress from congestive hepatopathy to fibrosis to cirrhosis with portal hypertension, and is associated with an increased risk of hepatocellular carcinoma (HCC). Current guidelines recommend intensive yearly folIow-up to assess FALD severity. However, the opted surveillance methods are unable to distinguish hepatic congestion from fibrosis or cirrhosis. Additionally, current literature provides limited evidence that FALD surveillance prevents adverse events. Intravoxel incoherent motion (IVIM) modelled diffusion-weighted imaging (DWI) allows for non-invasive differentiation between hepatic congestion and fibrosis, and has been suggested as a surveillance strategy in FALD. The objective of this study is to investigate the prognostic value of MRI-derived IVIM-modelled DWI in patients with a Fontan circulation. In a prospective study, 59 consecutive Fontan patients underwent hepatic MRI for IVIM-modelled DWI measurements. Apparent diffusion coefficient (ADC) and parameters of hepatic congestion—namely, the fast diffusion component (D fast , reflecting microperfusion-related water movement), the fraction of microperfusion (f fast , indicating the proportion of signal from microvascular perfusion), and fibrosis (slow diffusion component (D slow ), representing restricted water diffusion in dense tissue)—were measured. The association between DWI measurements and a composite endpoint of all-cause mortality and Fontan-related hospitalization was assessed using Cox-regression analyses. Mean cohort age was 19?±?8 years, 51% female. Mean ADC was (0.82?±?0.11)?×?10 ?3 mm 2 /s, D fast (20.74?±?6.60)?×?10 ?3 mm 2 /s, f fast 29?±?05%, and D slow (0.77?±?0.15)?×?10 ?3 mm 2 /s. During a median follow-up time of 9 (Q1-Q3: 5–11) years, the primary endpoint occurred in 33 patients (59%). We found no significant association between IVIM-modelled DWI parameters at inclusion and the composite primary endpoint at follow up (all p >?0.05). IVIM-modelled DWI addresses a key diagnostic challenge in FALD by enabling distinction between hepatic congestion and fibrosis. However, even after this distinction, we did not find a relationship between IVIM-modelled DWI and clinical outcomes. These findings, in combination with current literature data, which also fail to demonstrate a relation between other non-invasive FALD assessment tools and outcome, raise the question whether the recommended intensive FALD monitoring strategies are justified.

Introduction:
Fontan-associated liver disease (FALD) encompasses the spectrum of hepatic alterations in patients after Fontan palliation. FALD can progress from congestive hepatopathy to fibrosis to cirrhosis with portal hypertension, and is associated with an increased risk of hepatocellular carcinoma (HCC). Current guidelines recommend intensive yearly folIow-up to assess FALD severity. However, the opted surveillance methods are unable to distinguish hepatic congestion from fibrosis or cirrhosis. Additionally, current…

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