Skip to main content
Log in

MRI measured liver stiffness does not predict focal liver lesions after the Fontan operation

  • Original Article
  • Published:
Pediatric Radiology Aims and scope Submit manuscript

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Fontan F, Baudet E (1971) Surgical repair of tricuspid atresia. Thorax 26:240–248

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  2. Fredenburg TB, Johnson TR, Cohen MD (2011) The Fontan procedure: anatomy, complications, and manifestations of failure. Radiographics 31:453–463

    Article  PubMed  Google Scholar 

  3. 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

    PubMed  Google Scholar 

  4. 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

    Article  PubMed  PubMed Central  Google Scholar 

  5. Tellez L, Rodriguez-Santiago E, Albillos A (2018) Fontan-associated liver disease: a review. Ann Hepatol 17:192–204

    Article  PubMed  Google Scholar 

  6. 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

  7. Wallihan DB, Podberesky DJ (2013) Hepatic pathology after Fontan palliation: spectrum of imaging findings. Pediatr Radiol 43:330–338

    Article  PubMed  Google Scholar 

  8. 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

    Article  Google Scholar 

  9. 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

    Article  PubMed  Google Scholar 

  10. 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

    Article  PubMed  Google Scholar 

  11. 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

    Article  PubMed  Google Scholar 

  12. 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

    Article  CAS  PubMed  Google Scholar 

  13. 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

    Article  PubMed  Google Scholar 

  14. 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

    Article  PubMed  Google Scholar 

  15. 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

    Article  PubMed  Google Scholar 

  16. 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

    Article  PubMed  Google Scholar 

  17. 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

    Article  Google Scholar 

  18. 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

    Article  PubMed  Google Scholar 

  19. 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

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Jonathan R. Dillman.

Ethics declarations

Conflicts of interest

None

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

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

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00247-018-4264-z

Keywords

Navigation