166 Chapter 5 Association between plasma lysoGb3 and left ventricular mass and relative wall thickness on echocardiography In untreated FD patients, plasma lysoGb3 levels were significantly associated with the increase in left ventricular mass over time (indexed to body surface area, LVMi) on echocardiogram (p<0.001, n=192, median number of measurements: 1 per patient, range 1-12, fig 4a). Aside from lysoGb3 levels, having one or more cardiovascular risk factors (hypertension, smoking or obesity) was an independent risk factor for higher LVMi (p=0.003). Using the estimates in table 2, we can calculate that left ventricular mass of a patient with a plasma lysoGb3 value of 1 increases with 0.78g/m2 every year, compared to a yearly increase of 2.5g/m2 for a patient with a lysoGb3 of 100 nmol/L. Removing male patients with classical FD from the analyses did not change the outcome (p<0.001 for lysoGb3, p=0.005 for cardiovascular risk factor). Higher lysoGb3 levels were significantly associated with higher relative wall thickness (RWT), as a sign for concentric remodeling, at any age (p<0.001, fig. 4b). No association between the plasma lysoGb3 levels and the rate of increase of RWT was found. Female patients had a significantly higher RWT compared to male patients (p=0.04). Removing male patients with classical FD from the analyses did not change the outcome (p=0.001).
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