Abstract
Background
Focal liver lesions are common in children and adults after Fontan procedures.
Objective
To explore the relationship between liver shear stiffness, using magnetic resonance (MR) elastography, and the presence of focal liver lesions in patients after Fontan procedures (total cavopulmonary anastomosis).
Materials and methods
The retrospective study was approved by the institutional review board and the requirement for informed consent was waived. By searching institutional electronic medical records, we identified all patients with a history of Fontan palliation of congenital heart disease who had undergone same-day liver MR elastography and liver MRI without and with intravenous contrast material between January 2012 and December 2017. Using imaging reports, patients were placed into two groups: 1) no focal liver lesions and 2) one or more focal liver lesions. Patient age, sex, mean liver shear stiffness (kPa) and maximum single anatomical level liver shear stiffness (kPa) were recorded. The Mann-Whitney U test was used to compare age and liver stiffness between groups, while the Fisher exact test was used to assess the impact of gender on liver lesions.
Results
Forty-eight patients met study inclusion criteria; 33 (69%) had one or more focal liver lesions. The median age was 20.0 years (IQR [interquartile range]: 10.8-29.1 years) for patients without liver lesions and 19.9 years (IQR: 17.2-27.0 years) for patients with liver lesions (P=0.49). Eleven of 21 male patients (52.4%) had liver lesions compared to 22 of 27 female patients (81.5%) (P=0.058). Mean (4.62 kPa [IQR: 4.10–5.59 kPa] vs. 4.10 kPa [IQR: 3.44-4.80 kPa]; P=0.02) and maximum (5.53 kPa [IQR: 4.64-6.56 kPa] vs. 4.50 kPa [IQR: 3.82-5.35 kPa]; P=0.009) liver stiffness were significantly higher in patients without liver lesions as compared to patients with liver lesions.
Conclusion
Our study demonstrated a significant negative association between focal liver lesions and increased liver stiffness in patients following Fontan procedures.
Similar content being viewed by others
References
Fontan F, Baudet E (1971) Surgical repair of tricuspid atresia. Thorax 26:240–248
Fredenburg TB, Johnson TR, Cohen MD (2011) The Fontan procedure: anatomy, complications, and manifestations of failure. Radiographics 31:453–463
Hirsch JC, Goldberg C, Bove EL et al (2008) Fontan operation in the current era: a 15-year single institution experience. Ann Surg 248:402–410
Atz AM, Zak V, Mahony L et al (2017) Longitudinal outcomes of patients with single ventricle after the Fontan procedure. J Am Coll Cardiol 69:2735–2744
Tellez L, Rodriguez-Santiago E, Albillos A (2018) Fontan-associated liver disease: a review. Ann Hepatol 17:192–204
Goldberg DJ, Surrey LF, Glatz AC et al (2017) Hepatic fibrosis is universal following Fontan operation, and severity is associated with time from surgery: a liver biopsy and hemodynamic study. J Am Heart Assoc 6:pii:e004809
Wallihan DB, Podberesky DJ (2013) Hepatic pathology after Fontan palliation: spectrum of imaging findings. Pediatr Radiol 43:330–338
Wells ML, Hough DM, Fidler JL et al (2017) Benign nodules in post-Fontan livers can show imaging features considered diagnostic for hepatocellular carcinoma. Abdom Radiol (NY) 42:2623–2631
Ghaferi AA, Hutchins GM (2005) Progression of liver pathology in patients undergoing the Fontan procedure: chronic passive congestion, cardiac cirrhosis, hepatic adenoma, and hepatocellular carcinoma. J Thorac Cardiovasc Surg 129:1348–1352
DiPaola FW, Schumacher KR, Goldberg CS et al (2017) Effect of Fontan operation on liver stiffness in children with single ventricle physiology. Eur Radiol 27:2434–2442
Wallihan DB, Podberesky DJ, Marino BS et al (2014) Relationship of MR elastography determined liver stiffness with cardiac function after Fontan palliation. J Magn Reson Imaging 40:1328–1335
Wang D, Marshall D, Veldtman G et al (2018) Intrahepatic cholangiocarcinoma after Fontan procedure in an adult with visceral heterotaxy. Pathol Res Pract 214:914–918
Daniels CJ, Bradley EA, Landzberg MJ et al (2017) Fontan-associated liver disease: proceedings from the American College of Cardiology Stakeholders Meeting, October 1 to 2, 2015, Washington DC. J Am Coll Cardiol 70:3173–3194
Nandwana SB, Olaiya B, Cox K et al (2018) Abdominal imaging surveillance in adult patients after Fontan procedure: risk of chronic liver disease and hepatocellular carcinoma. Curr Probl Diagn Radiol 47:19–22
Kim YS, Jang YN, Song JS (2018) Comparison of gradient-recalled echo and spin-echo echo-planar imaging MR elastography in staging liver fibrosis: a meta-analysis. Eur Radiol 28:1709–1718
Serai SD, Dillman JR, Trout AT (2017) Spin-echo echo-planar imaging MR elastography versus gradient-echo MR elastography for assessment of liver stiffness in children and young adults suspected of having liver disease. Radiology 282:761–770
Wagner M, Besa C, Bou Ayache J et al (2016) Magnetic resonance elastography of the liver: qualitative and quantitative comparison of gradient echo and spin echo echoplanar imaging sequences. Investig Radiol 51:575–581
Trout AT, Sheridan RM, Serai SD et al (2018) Diagnostic performance of MR elastography for liver fibrosis in children and young adults with a spectrum of liver diseases. Radiology 287:824–832
Sinclair M, Schelleman A, Sandhu D, Angus PW (2017) Regression of hepatocellular adenomas and systemic inflammatory syndrome after cessation of estrogen therapy. Hepatology 66:989–991
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflicts of interest
None
Rights and permissions
About this article
Cite this article
Diaz, E.S., Dillman, J.R., Veldtman, G.R. et al. MRI measured liver stiffness does not predict focal liver lesions after the Fontan operation. Pediatr Radiol 49, 99–104 (2019). https://doi.org/10.1007/s00247-018-4264-z
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00247-018-4264-z