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Objectives: To explore the utility of liver T1 mapping in Fontan patients and its correlation to magnetic resonance elastography (MRE)-derived liver stiffness., Background and Aims: Liver disease is a major long-term extra cardiac complication in the Fontan population. MRE is frequently used to quantify liver stiffness in Fontan patients; however, it has certain limitations. Native T1 mapping by cardiac magnetic resonance (CMR) is useful in assessment of cardiac fibrosis, but its potential in evaluating liver fibrosis and its correlation to MRE-derived liver stiffness in Fontan patients have not been reported., Methods: Fontan patients who underwent CMR and MRE were included. Liver Native T1, extracellular volume (ECV) and delta coefficients were measured and correlated with MRE-derived liver stiffness in all Fontan patients. Native liver T1 in Fontan patients were compared to normal controls with biventricular circulation and no known liver disease., Results: A total of 17 Fontan patients and 7 normal controls were included in this study. Fontan patients had significantly higher liver native T1 (690 ± 41 ms vs 620 ± 35 ms; p < 0.001) as compared to controls. There was strong positive correlation between MRE derived liver stiffness and liver native T1 (r = 0.81, p < 0.001)., Conclusions: Liver native T1 was significantly elevated in Fontan patients compared to controls and strongly correlated with MRE-derived liver stiffness. This technique may prove to be a useful noninvasive imaging biomarker for assessing liver fibrosis in the Fontan population.

The goal of this study was to assess the ability of quantitative T1 cardiovascular magnetic resonance (CMR) imaging to calculate liver extracellular volume (ECV) in patients with varying degrees of congestive hepatopathy (CH). T1 measurements and ECV calculations were performed retrospectively in three cohorts of patients: normal cardiac function, tetralogy of fallot (TOF) repair and Fontan palliation. All CMR studies included modified look-locker inversion recovery (MOLLI) T1 mapping scans performed pre- and post-injection of a gadolinium-based contrast agent (GBCA). Pixel intensity data were manually collected from images of the liver and cardiac blood pool to determine contrast-induced changes in T1 for liver and blood. These data were then used to compute liver ECV. 172 subjects were included in the study. Of these, 140 subjects were normal cardiac function patients, 16 were TOF repair patients and 16 patients were with Fontan palliation. A statistically significant difference in both the liver native T1 and ECV measurements was found between patients with normal cardiac function vs. Fontan palliation patients (p < 0.01). Our data indicate that measuring T1 maps both pre- and post-GBCA injection within CMR scan session can be used to follow progression of liver fibrosis. This technique has the potential to improve diagnosis and treatment of patients with chronic liver disease and liver fibrosis.

Purpose: The purpose of this study is determine if both liver T2* and cardiac T2* can be measured on a single breath-hold acquisition., Materials and Methods: For this IRB-approved retrospective study, 137 patients with dedicated Cardiac MRI and Liver MRI examinations obtained sequentially on 1.5T scanners and on the same day were included for analysis. Both the cardiac and liver MRI examinations utilized GRE sequences for quantification of tissue iron. Specifically, T2* was measured using an 8-echo, multi-echo gradient echo single breath-hold sequence. Liver T2* was measured in a blinded manner on images from each of the cardiac and dedicated liver MRI examinations and were correlated. Bland-Altman difference plot was used to assess mean bias., Results: 137 examinations from 93 subjects met inclusion criteria. 10 examination pairs were excluded because the first echo time (TE) on the cardiac MRI was insufficiently short for the very high liver iron content. After exclusion, 127 studies from 89 subjects (67.4% males) were included in the final analysis. The mean subject age (± standard deviation) was 11.5 ± 7.5 years (range 0-29.3 years; median 10.5 years). Mean liver T2* measured on cardiac MRI was 8.3 ± 7.7 ms and mean liver T2* measured on dedicated liver MRI was 7.8 ± 7.4 ms (p < 0.001). There was strong positive correlation between the two liver T2* measurements (r = 0.989, p < 0.0001; 95% CI 0.985-0.992). With the exception of borderline outliers, all values fell within two standard deviations on the Bland-Altman difference plots, with a mean bias of 0.5 ms (range - 1.8 to + 2.7 ms)., Conclusion: In most patients with suspected or known iron overload, a single breath-hold GRE sequence may be sufficient to evaluate the iron concentration (T2*) of both the myocardium and the liver.

The cardiac disease ubiquitously associated in Duchenne Muscular Dystrophy (DMD) has traditionally been considered a progressive dilated cardiomyopathy (DCM). However, left ventricular (LV) dilatation as measured with cardiac MRI has not been a consistent finding in this population, even as circumferential strain (ε(cc)) declines with advancing disease. We hypothesized that a distinct pattern of changes in LV geometry, during the course of ε(cc) decline, distinguishes DMD associated heart disease from DCM. Using CMR, LV end-diastolic volume (EDV), mass (LVM), ejection fraction, ε(cc) and myocardial delayed enhancement (MDE) were determined in DMD patients and normal control subjects. The LV Remodeling Index (LVRI) was calculated as the ratio of LV Mass to Volume (LVM/EDV). Statistical comparisons between all LV parameters and genotype were also performed. Median LVRI in DMD (n = 127) and control subjects (n = 41) were different (0.75 vs. 0.65, P = 0.0150) but within normal range. Furthermore, the median LVRI in DMD boys with reduced LV systolic function was significantly reduced compared to those with normal LV systolic function (0.64 vs. 0.75, P = 0.0974). However, the presence of MDE was associated with a lower median LVRI (0.57 vs. 0.76, P = 0.0471). Regression analysis showed no significant correlation between ε(cc) and LVRI (r = -0.03). The LVRI of DMD patients is unexpectedly normal and not correlated with ε(cc.) Based on these findings, DMD-associated heart disease exhibits a unique remodeling pattern distinct from DCM.