129 Echocardiographic changes in Fabry disease LVMi and LAVI In men and women with FD, LVMi and LAVI increased significantly over time, whilst remaining stable in healthy subjects (table 2, figure 1C-1D). When comparing men and women with FD, the increase in LVMi was greater in men compared to women (FD: men minus women β=13.5 g/m2 per decade; 95%-CI 7.8-19.1). The change over time in LAVI did not differ between men and women with FD (FD: men minus women β= 3.2 ml/m2 per decade; 95%-CI -0.77.1), but the value for LAVI was overall higher in FD men compared to women with FD throughout follow-up (figure 1D). Compared to healthy controls, men with FD had a higher LVMi from 30 years onwards, whilst in women this point was reached from 40 years onwards. The absolute value for LAVI was significantly different in men with FD compared to healthy men from 50-59 years onwards. For women the absolute values for LAVI were not significantly different between FD patients and healthy control subjects (figure 2D, supplemental table 1). LVMi showed median values above the normal range from age 40 years onwards in men with FD and from age 50 years onwards in women with FD. Values outside the reference range for LAVI (> 34 ml/m2) were present approximately ten years later (figure 2C-2D, supplemental table 1). Functional echocardiographic features E/e’ increased considerably with time in men and women with FD, in contrast to healthy individuals in whom E/e’ was similar at all ages (table 2) (men: FD minus healthy controls β= 2.7 per decade; 95%- CI 1.6-3.7 and women: FD minus healthy controls β= 1.3 per decade; 95%-CI 0.4-2.2). The increment in E/e’ over time was greater in men with FD compared to women with FD (FD: men minus women β= 1.1 per decade; 95%-CI 0.2-2.0) (figure 1E). When comparing age subgroups, the absolute values for E/e’ in men with FD are significantly higher as compared to healthy control subjects throughout adult life, while this parameter differs significantly between women with FD compared to healthy control subjects from 50 years onwards. Values for E/e’ increase to levels outside of the relevant references range (> 14) [27] in men with FD from the age of 50 years onwards, while this occurs approximately a decade later in women with FD (figure 2E, supplemental table 1). Grading of the LV diastolic function was possible for 84 out of 92 FD patients (91%) using the last available echocardiograms (echocardiography images 4
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