Temporal changes in liver stiffness after Fontan operation: Results of serial magnetic resonance elastography

Alexander Egbe, William R. Miranda, Heidi M. Connolly, Arooj R. Khan, Mohamad Al-Otaibi, Sudhakar K. Venkatesh, Douglas Simonetto, Patrick Kamath, Carole Warnes

Research output: Contribution to journalArticlepeer-review

19 Scopus citations


Background: The relationship between temporal progression of magnetic resonance elastography derived liver stiffness (MRE-LS) and progression of Fontan associated liver disease (FALD) is unknown. To assess this relationship, we hypothesized that progression of MRE-LS correlated with progression FALD severity and clinical outcomes. Methods: Retrospective review of Fontan patients who had >1 liver MRE, 2010–2016. Annual change in MRE-LS was the quotient of the difference between baseline and subsequent MRE-LS, and the interval between scans. Results: 22 patients were enrolled; median age 29(19–38) years, 14 (64%) males and 10 (46%) with atriopulmonary Fontan. Baseline and subsequent MRE-LS values were 5.4 ± 1.1 kPa and 5.8 ± 0.9 kPa" for clarity, interval between scans was 25 ± 5 months, and annual change in MRE-LS was 0.3 ± 0.2 kPa. Temporal change in MRE-LS correlated with temporal changes in model for end-stage liver disease (MELD) score (r = 0.84, p < 0.001) and model for end-stage liver disease excluding international normalized ratio (MELD-XI) score (r = 0.75, p = 0.001). The study cohort was divided into 2 groups using the mean annual change in MRE-LS as the cut point. Groups A and B comprised of patients with annual increase in MRE-LS ≥0.3 kPa (n = 6) and <0.3 kPa (n = 16) respectively. Composite adverse event endpoint (death, heart–liver transplant listing, palliative care, hospitalization, paracentesis) was more common in Group A (4 of 6, 67%) compared to Group B (3 of 16, 19%), p = 0.13 although this did not reach statistical significance due to small sample size. Conclusions: Progression of MRE-LS correlated with clinical deterioration as measured by worsening liver disease severity scores and the occurrence of adverse events.

Original languageEnglish (US)
Pages (from-to)299-304
Number of pages6
JournalInternational Journal of Cardiology
StatePublished - May 1 2018


  • Cirrhosis
  • Fontan associated liver disease
  • Liver stiffness
  • Magnetic resonance elastography

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine


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