Diagnostic performance of native T1 in grading histologically proven myocardial glycosphingolipid accumulation in Fabry disease

Figures

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  Fig. 1. nT1 values assessment and morphometric analysis of EMB specimens. Upper row: nT1 values were calculated per plane (on the left, as the area included between epicardial (green) and endocardial (red) contours), in a region of interest drawn within the interventricular septum (in the center image) and per segment (basal anteroseptal (segment 2) and inferoseptal (segment 3)). Lower row: Assessment of cell area occupied by GSL vacuoles (a), cardiomyocytes diameter measurement (b) and fibrosis amount calculation (c)

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  Fig. 2. Bullseye view of basal and midventricular planes. Global nT1 (a), T2 (b) and ECV (c) mean values and inferoseptal and anteroseptal nT1 (a), T2 (b) and ECV (c) mean values for all patients

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  Fig. 3. Box plot graphs of nT1 values measured at AHA segment 9, midventricular plane and mIVS ROI according to GSL-A and LVH severity. Distribution of nT1 values for different degrees of GSL-A (upper row) and LVH (lower row). In all the box plots, the top of the box represents the third quartile and the bottom the first quartile. The horizontal line represents the median for the entire cohort. The whiskers go from each quartile to the minimum or maximum

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  Fig. 4. Correlation between nT1 values measured at midventricular plane, AHA segment 9, mIVS ROI vs GSL-A

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  Fig. 5. Diagnostic performance of nT1 values measured at midventricular plane, AHA segment 9, mIVS ROI in detecting patients with severe GSL-A. ROC curve illustrates the diagnostic performance of nT1 measured at the best three myocardial sites to identify patients with severe GSL-A

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  Fig. 6. Line graph of mean nT1 and T2 values among the GSL-A groups. The graph shows a gradual decline in nT1 values along with growing GSL-A degree (orange line), with a plateau in mild-moderate groups, whereas matched T2 values show an opposite trend (blue line)