84 Chapter 3 mild phenotype (severely affected patients have by then died or received a pacemaker) could incorrectly suggest that with advanced age, male patients have a similar electrocardiographic phenotype as female FD patients. Second, in FD patients, changes in electrophysiological parameters reflect time- dependent alterations of each parameter (longitudinally collected ECGs). However, for the control subjects, the data were obtained in a cross-sectional study and no changes within the included subjects can be analyzed. Third, the gross majority of the included patients were treated with enzyme replacement therapy (ERT) for at least part of the follow-up and the electrophysiological changes depicted do not represent the natural disease course. This effect of treatment (ERT) on the electrophysiological parameters cannot be studied given the small untreated patient sample, the treatment indication bias and the vast difference in age at which treatment was started between patients. Previous studies indicated that cardiac disease may progress despite ERT [19, 42], this is confirmed by the current study. The data from the current study, can, however, be used to analyze the effect of new FD therapies to see if they outperform current treatment in preventing progression of cardiac disease.
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