85 ECG changes in Fabry disease Conclusions In FD, several ECG parameters show progressive alterations during adult life. The frontal QRS-axis is already significantly different in both male and female FD patients aged 18-29 years and is thus the earliest marker of cardiac disease identified in this study. For male patients this is also true for the Cornell index, with female patients following a decade later. For the other ECG parameters, specifically the rate of change throughout adulthood, more than the absolute values, is grossly different in FD patients from that in apparently healthy individuals. Tracking the rate of change could be a good way to detect disease progression in early adulthood, guiding treatment initiation in those that show significant changes. At later disease stages the absolute values for all ECG parameters, except for PR-interval, become abnormal, in comparison to both the controls and the reference values. All studied ECG parameters showed a positive correlation with left ventricular mass index on CMR. P-wave duration, QRSduration, QTc, Cornell index and spatial QRS-T angle were different in patients with cardiac fibrosis compared to those without and the spatial QRS-T angle could be used to identify those requiting CMR follow up of fibrosis development. 3
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