132 Chapter 4 Relationship of echocardiographic features and cardiac enzymes in FD patients 164 NT-plasma NT-proBNP and 111 hs-TnT values were available to be linked to data from the concomitant echocardiograms. There was a significant but moderate correlation (R= between 0.50- 0.69) between the height of the plasma NT-proBNP and hs-TnT levels and LVMi, E/e’ and GLS values (supplemental figure 6). Echocardiographic features and development of atrial fibrillation in FD patients Seven patients developed a MACE after the first echocardiography, this number was too small to study the relationship between the echocardiographic markers and MACE. Fourteen patients developed AF after the first echocardiogram. For all the 5 parameters analyzed a higher value was associated with AF (Hazard ratio’s (HR) between 1.02 and 1.50). The strongest relation was for RWT and IVSd: patients with a higher RWT on the first echocardiogram had a HR of 1.50 (95%CI: 1.25- 1.80) for the development of AF, for a higher IVSd this was 1.31 (95%CI: 1.15-1.49). See supplemental table 4 for the results on the Cox regression analyses. Discussion This is the first long-term longitudinal study that assessed the evolution of echocardiographic features during adult life in classical FD patients versus healthy control subjects. It shows that the progressivecourse of the disease can be documented with serial echocardiography. For early detection of cardiac disease in FD patients three markers appear particularly useful: the morphological markers RWT and IVSd and the functional marker E/e’. RWT is higher in both male and female FD patients from the age of 30 years onwards and potentially even earlier, but the number of patients aged 18-29 years old was too small to confirm this. It is noteworthy to emphasize that RWT and IVSd in early adulthood were only higher in FD patients when compared to age and sex control specific ranges, not when compared to the usual reference values. Thus, the use of age and sex specific reference ranges is key for detecting the early, subtle changes in these parameters (supplemental table 1). E/e’, an echocardiographic parameter of left atrial pressure, is a particularly relevant marker of early disease in male patients with classical FD, in whom it is already significantly increased in the first decade of adult life. E/e’ is significantly associated with both plasma NT-proBNP and troponin T, showing its potential
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