167 Plasma globotriaosylsphingosine and the natural Fabry disease course Figure 4: (a) Relation between lysoGb3 and the increase (slope) of left ventricular mass (LVMi) over time in untreated FD patients. Reference ranges of normal LVMi are 49-115 g/m2 for males and 43-95 for females,>149g/m2 is considered grossly abnormal in males and >122 g/m2 for females [22]. (b) Association of plasma lysoGb3 with RWT on echocardiography in untreated Fabry patients. RWT was higher in patients with higher lysoGb3 values, but there was no difference in slope over time. Values above >0.42 suggest concentric remodeling [22]. All analyses are performed with actual plasma lysoGb3 levels (after log10 transformation). LysoGb3 levels are grouped for visualization purposes only as described in the legend. *Patient suffered severe cardiac decompensation between first and last assessment. Patient was repeatedly admitted to ICU after last assessment and passed away 2 years later (due to heart failure). In addition, quality of the echocardiograms in this patient were described as suboptimal. Removing the patient from analyses did not change the outcome of the analysis. 5
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