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Myocardial fibrosis, diastolic dysfunction and elevated liver stiffness in the Fontan circulation

Tarek Alsaied1, Ryan A Moore2, Sean M Lang2

  • 1Department of Pediatrics, The Heart Institute, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA tarek.alsaied@cchmc.org.

Open Heart
|October 28, 2020
PubMed

Insights

Myocardial fibrosis in Fontan circulation patients is linked to diastolic dysfunction and liver stiffness. This suggests a systemic profibrotic state affecting organs in adolescents and young adults.

Area of Science:

  • Cardiology
  • Hepatology
  • Biomarkers

Background:

  • Single ventricle physiology often leads to diastolic dysfunction and hepatic fibrosis in Fontan circulation patients.
  • The connection between poor hemodynamics and end-organ fibrosis in young Fontan patients remains unclear.

Purpose of the Study:

  • To investigate the relationship between myocardial fibrosis, liver stiffness, and serum fibrosis biomarkers in adolescents and young adults with Fontan circulation.

Main Methods:

  • Prospective observational study including cardiac catheterization and cardiac MRI with T1 mapping for extracellular volume (ECV) measurement.
  • Hepatic magnetic resonance elastography assessed liver stiffness; serum biomarkers (MMPs, TIMPs) were measured.

Main Results:

  • A significant correlation was found between ECV and systemic ventricular end-diastolic pressure (r=0.42, p=0.03) and liver stiffness (r=0.45, p=0.05).
  • Elevated ECV, MMPs, and TIMPs were associated with increased liver stiffness, indicating a profibrotic state.

Conclusions:

  • Cardiac MRI evidence of myocardial fibrosis is associated with diastolic dysfunction, increased liver stiffness, and elevated fibrosis biomarkers in Fontan patients.
  • These findings suggest a systemic profibrotic environment impacting end-organs in some Fontan circulation patients.
Abstract

Keywords:
cardiac catheterisationdiagnostic imagingmagnetic resonance imaging

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