The impact of Wilson disease on myocardial tissue and function: a cardiovascular magnetic resonance study

Figures

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  Fig. 1. Flowchart of patients inclusion. Flowchart of Wilson Disease (WD) included in study. WD-neuro−—Wilson Disease with primarily hepatic manifestation and without neurological symptoms, WD-neuro + —Wilson Disease with neurological symptoms

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  Fig. 2. Cardiac structural and functional changes in patients with Wilson Disease with and without neurological symptoms. Comparison of Wilson Disease (WD) patients without neurological symptoms (WD-neuro−), with neurological symptoms (WD-neuro+) and healthy controls: Septum (mm) (a); Left ventricular global circumferential strain (LV-GCS (%)) (b), global native T1 time (ms) (c) and extracellular volume fraction (ECV) (%) (d) in 1.5-T CMR. Differences between all three groups were calculated using 2-way ANOVA or Kruskal–Wallis test, * p < 0.05 vs. controls; **p < 0.01 vs. controls; $p < 0.05 vs. WD neuro− (t-test)

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  Fig. 3. Images of left ventricular global circumferential strain in patients with Wilson. Disease with and without neurological symptoms. Images of strain using fSENC: left ventricular (LV) two- (2-CH), three- (3-CH) and four- (4-CH) chamber views at end-systolic phase used to calculate LV global circumferential strain (GCS). Upper row—controls (male, GCS − 21.5%), middle row—Wilson Disease (WD) patients without neurological symptoms (WD-neuro−) (male, GCS − 20.5%), lower row—WD patients with neurological symptoms (WD-neuro+) (male, GCS − 16.8%). Color maps range from violet indicating strain up to − 32% to red indicating a severely reduced strain (+ 12%) (as shown in color scale)

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  Fig. 4. Cine images and native T1 MOLLI in patients with Wilson Disease (WD) with and without neurological symptoms. Images of left ventricular in Wilson Disease (WD) patients: controls (a) (male, septum 8 mm, lateral wall 6 mm), WD patients without neurological symptoms (WD-neuro−) (b) (male, septum 9 mm, lateral wall 7 mm) and WD patients with neurological symptoms (WD-neuro+) (male, septum 11 mm, lateral wall 9 mm) (c). Images of native T1 times in controls (d) (female, global native T1 times 1014 ms), WD-neuro− (e) (female, global native T1 times 1055 ms) and WD-neuro+ (f) (male, global native T1 times 1070 ms). Colors (1.5 T MRI); color scale displays T1 values (ms)

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  Fig. 5. Images of left ventricular global longitudinal strain in patients with Wilson Disease (WD) with and without neurological symptoms. Images of strain using fSENC: left ventricular (LV) short-axis views at LV basal, mid-ventricular (mid) and apical level used to calculate global LV longitudinal strain (GLS). Upper row—controls (male, GLS − 21.8%), middle row—Wilson Disease (WD) patients without neurological symptoms (WD-neuro−) (male, GLS − 17.3%), lower row—WD patients with neurological symptoms (WD-neuro+) (male, GLS − 16.0%). Color maps range from violet indicating strain up to − 32% to red indicating a severely reduced strain (+ 12%) (as shown in color scale)

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  Fig. 6. Representative images of late gadolinium enhancement (LGE) in patients with Wilson Disease (WD) with and without neurological symptoms. Representative images of LGE results in WD patients at the inferior insertion point of the right ventricular free wall (a), as mid-wall sign (b) and subepicardial inferolateral (c, d)

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  Fig. 7. Representative images of extracellular volume fraction (ECV) in patients with Wilson Disease (WD) with and without neurological symptoms. Representative images of extracellular volume (ECV%) in WD patients without (WD neuro−) (male, ECV = 23.5%) (a) and WD patients with neurological symptoms (WD-neuro+) (female, ECV = 30.4%) (b). Color maps range from blue indicating ECV 1–10% to red indicating severely elevated ECV up to 50% (as shown in color scale)