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J Cardiovasc Magn Reson. 2019 Jul 15;21(1):39. doi: 10.1186/s12968-019-0545-4.

Increased extracellular volume in the liver of pediatric Fontan patients.

Author information

1
Department of Pediatrics, Division of Cardiology, The Hospital for Sick Children, University of Toronto, Ontario, Canada.
2
Division of Radiology and Nuclear medicine, Pediatric section, Rikshospitalet, Oslo University Hospital, Oslo, Norway.
3
Department of Pediatrics, Division of Cardiology, Stollery Children's Hospital, University of Alberta, Edmonton, Alberta, Canada.
4
Department of Diagnostic Imaging, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
5
Knight Cardiovascular Institute, Division of Cardiovascular Medicine, Oregon Health and Science University, Portland, Oregon, USA.
6
Department of Pediatrics, Division of Cardiology, The Hospital for Sick Children, University of Toronto, Ontario, Canada. lars.grosse-wortmann@sickkids.ca.
7
Department of Diagnostic Imaging, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. lars.grosse-wortmann@sickkids.ca.

Abstract

BACKGROUND:

Patients with single ventricle physiology are at increased risk for developing liver fibrosis. Its extent and prevalence in children with bidirectional cavopulmonary connection (BCPC) and Fontan circulation are unclear. Extracellular volume fraction (ECV), derived from cardiovascular magnetic resonance (CMR) and T1 relaxometry, reflect fibrotic remodeling and/or congestion in the liver. The aim of this study was to investigate whether pediatric patients with single ventricle physiology experience increased native T1 and ECV as markers of liver fibrosis/congestion.

METHODS:

Hepatic native T1 times and ECV, using a cardiac short axis modified Look-Locker inversion recovery sequence displaying the liver, were measured retrospectively in children with BCPC- and Fontan circulations and compared to pediatric controls.

RESULTS:

Hepatic native T1 time were increased in Fontan patients (n = 62, 11.4 ± 4.4 years, T1 762 ± 64 ms) versus BCPC patients (n = 20, 2.8 ± 0.9 years, T1 645 ± 43 ms, p = 0.04). Both cohorts had higher T1 than controls (n = 44, 13.7 ± 2.9 years, T1 604 ± 54 ms, p < 0.001 for both). ECV was 41.4 ± 4.8% in Fontan and 36.4 ± 4.8% in BCPC patients, respectively (p = 0.02). In Fontan patients, T1 values correlated with exposure to cardiopulmonary bypass time (R = 0.3, p = 0.02), systolic and end diastolic volumes (R = 0.3, p = 0.04 for both) and inversely with oxygen saturations and body surface area (R = -0.3, p = 0.04 for both). There were no demonstrable associations of T1 or ECV with central venous pressure or age after Fontan.

CONCLUSION:

Fontan and BCPC patients have elevated CMR markers suggestive of hepatic fibrosis and/or congestion, even at a young age. The tissue changes do not appear to be related to central venous pressures.

TRIAL REGISTRATION:

Retrospectively registered data.

KEYWORDS:

Cardiovascular magnetic resonance; Fontan circulation; Liver cirrhosis; Single ventricle; T1 mapping

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