• Djoeke Wolff, Joost P van Melle, Hildebrand Dijkstra, Beatrijs Bartelds, Tineke P Willems, Hans Hillege, Aad P van den Berg, Tjark Ebels, Paul E Sijens, and Rolf M F Berger.
• Center for Congenital Heart Diseases, Department of Pediatric Cardiology/Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, The Netherlands. Electronic address: d.wolff@umcg.nl.
• Int. J. Cardiol. 2016 Jan 1; 202: 595-600.

BackgroundPatients with a Fontan circulation tend to develop liver fibrosis, liver cirrhosis and even hepatocellular carcinoma. The aim of this study is to use the magnetic resonance technique diffusing-weighted imaging (DWI) for detecting liver fibrosis/cirrhosis in Fontan patients and to establish whether DWI results are associated with functional aspects of the Fontan circulation.MethodsIn a cross-sectional study, 59 Fontan patients were evaluated by liver DWI. The association between apparent diffusion coefficients (ADC) and patient characteristics, laboratory measurements and functional aspects of the Fontan circulation (NYHA class, maximum oxygen uptake during exercise and cardiac index) was assessed.ResultsLiver ADC values were low (0.82×10(-3)±0.11×10(-3) mm2/s) compared with literature values for healthy volunteers and correlated negatively with calculated liver fibrosis/cirrhosis scores (Fib-4 score, p=0.019; AST/ALT ratio, p=0.009) and gamma-glutamyl transferase (p=0.001). Furthermore, ADC values correlated negatively with follow-up duration (p<0.001) and positively with cardiac index (p=0.019). No correlation between ADC values and exercise tests was found. In multivariable analysis, the ADC values were independently correlated with follow-up duration after Fontan completion.ConclusionsThe results of the current study suggest that progressive liver damage due to chronic congestion and potential hypoperfusion is reflected in the liver ADC values in Fontan patients. This study highlights that liver damage in the context of the Fontan circulation might be far more common than previously thought, and that the implementation of liver assessment in the routine follow-up of Fontan patients is recommendable.Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

28 keywords...

hide…