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

Increased extracellular volume in the liver of pediatric Fontan patients

Charlotte de Lange  1   2 , Marjolein J E Reichert  1 , Joseph J Pagano  3 , Mike Seed  1   4 , Shi-Joon Yoo  1   4 , Craig S Broberg  5 , Christopher Z Lam  4 , Lars Grosse-Wortmann  6   7
Affiliations

Increased extracellular volume in the liver of pediatric Fontan patients

Charlotte de Lange et al. J Cardiovasc Magn Reson. .

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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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Hepatic Native T1. Native T1 source image in short-axis orientation demonstrating the heart and upper part of the abdomen. Regions of interest (ROIs) depict liver regions near diaphragm (#1, orange), central (#2, pink) and caudal (#3, yellow). The red ROI samples signal in the blood pool of a liver vein and the white ROI (#5) in the ventricular cavity. Stomach (st)
Fig. 2
Fig. 2
Boxplot of native T1 values. Boxplot of native T1 values in the Fontan, bidirectional cavopulmonary connection (BCPC) and control groups
Fig. 3
Fig. 3
Reproducibility of T1 and ECV. Bland Altman plots for intra and inter observer agreement for measurements of native T1 (upper row) and Extracellular volume fraction (ECV, lower row) for the average of ROIs 1–3
See this image and copyright information in PMC

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