Increased extracellular volume in the liver of pediatric Fontan patients

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. Electronic supplementary material The online version of this article (10.1186/s12968-019-0545-4) contains supplementary material, which is available to authorized users.

Hepatic magnetic resonance T1-mapping and extracellular volume fraction compared to shear-wave elastography in pediatric Fontan-associated liver disease

“…The elevated hepatic T1, extracellular volume fraction, and elastography values confirmed previous findings from retrospective MR studies in adults and children with Fontan [18,21,22] and studies on US and MR elastography after Fontan completion [19,20,27,28]. The advantage of our study was the representative, unselected, relatively large national cohort of adolescents with Fontan circulation.…”

“…US shear-wave elastography is simpler and less resource-demanding than MR, and is routinely used to assess adult chronic liver disease [16,17]. Few studies have employed hepatic MR T1 mapping or elastography to evaluate fibrosis or congestion in people with Fontan circulation [18][19][20][21][22]. We hypothesized that adolescents with Fontan circulation might display elevations in fibrosis markers, based on MR T1 mapping, extracellular volume fraction, and US shear-wave elastography.…”

Detection of Passive Liver Congestion in Pericarditis Patients With Constrictive Physiology Using MR Relaxometry of the Liver.

“…In contrast, relaxometry of the liver in cardiac patients has received limited interest. Preliminary results suggested that mapping of the liver may be useful in patients with tetralogy of Fallot and in Fontan circulation [15,16]. Our ndings not only endorse those ndings but suggest a broader use of liver MR relaxometry offering valuable information in cardiac conditions which affect the liver with potential deleterious consequences, such as cardiac cirrhosis.…”

Potential of Liver T₁ Mapping for the Detection of Fontan-associated Liver Disease in Adults

“…16,28,29 Alternatively, only a few studies have reported the evaluations of T 1 mapping for FALD in children and adolescents. 17,18 They demonstrated significantly elevated liver native T 1 values in Fontan patients than normal controls, and a strong correlation with MR elastography-derived liver stiffness. 17,18 However, whether T 1 mapping can differentiate congestion from fibrosis remains unclear.…”

“…T 1 mapping is a novel MRI technology to quantify the diffuse damage/fibrosis/congestion/edema/inflammation of parenchyma in organs such as the liver, heart, and pancreas. 15,16 Liver data in cases of congenital heart disease (CHD) are particularly lacking, with only a few prior studies in children and adolescents with Fontan circulation that reported the potential value of the liver native T 1 values as a screening tool; 17,18 however, there is no data for adults with Fontan circulation. Furthermore, the correlations between the liver disease biomarkers and T 1 values remain unknown.…”