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 Sequeira Kamath, Carole Warnes

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

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)
JournalInternational Journal of Cardiology
DOIs
StateAccepted/In press - Jan 1 2018

Fingerprint

Fontan Procedure
Elasticity Imaging Techniques
Liver
End Stage Liver Disease
Liver Diseases
Paracentesis
International Normalized Ratio
Palliative Care
Sample Size
Hospitalization

Keywords

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

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

Cite this

Temporal changes in liver stiffness after Fontan operation : Results of serial magnetic resonance elastography. / Egbe, Alexander; Miranda, William R.; Connolly, Heidi M.; Khan, Arooj R.; Al-Otaibi, Mohamad; Venkatesh, Sudhakar K; Simonetto, Douglas; Kamath, Patrick Sequeira; Warnes, Carole.

In: International Journal of Cardiology, 01.01.2018.

Research output: Contribution to journalArticle

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abstract = "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.",
keywords = "Cirrhosis, Fontan associated liver disease, Liver stiffness, Magnetic resonance elastography",
author = "Alexander Egbe and Miranda, {William R.} and Connolly, {Heidi M.} and Khan, {Arooj R.} and Mohamad Al-Otaibi and Venkatesh, {Sudhakar K} and Douglas Simonetto and Kamath, {Patrick Sequeira} and Carole Warnes",
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T2 - Results of serial magnetic resonance elastography

AU - Egbe, Alexander

AU - Miranda, William R.

AU - Connolly, Heidi M.

AU - Khan, Arooj R.

AU - Al-Otaibi, Mohamad

AU - Venkatesh, Sudhakar K

AU - Simonetto, Douglas

AU - Kamath, Patrick Sequeira

AU - Warnes, Carole

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N2 - 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.

AB - 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.

KW - Cirrhosis

KW - Fontan associated liver disease

KW - Liver stiffness

KW - Magnetic resonance elastography

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