64 Chapter 3 stage are important to be able to limit further progression of cardiac disease in FD patients. ECG parameters might be suitable to track this progression. Previous cross-sectional and longitudinal studies with a relatively small sample size show that early ECG abnormalities in FD patients include a short PR-interval and bradycardia [18, 19]. A long P-wave duration, prolonged QRS-duration, QTc, high QRS-amplitude, T-wave inversion and left frontal QRS- axis deviation may occur in later disease stages [19-21]. There are no longitudinal studies to show electrophysiological development over time in different patient groups (e.g., men versus women with FD) and how this differs from age related changes in ECG parameters in healthy individuals. This knowledge is needed to guide timing of treatment initiation (especially in women). But also to be able to detect the effect of (new) FD treatments on cardiac disease progression, since overt clinical complications take decades to develop [22, 23], far surpassing the duration of clinical trials. We hence conducted a retrospective study in the FD patient cohort under followup at the Amsterdam University Medical Centres (AUMC), to establish the course of electrophysiological parameters in male and female FD patients, to compare them to apparently healthy control subjects and to study their relationship to left ventricular mass and the presence of fibrosis on CMR. This study is unique in terms of both sample size and length of systematic ECG follow-up of FD patients. Primary aims of the study are: 1. Describing the evolution of alterations in ECG parameters in patients with classical FD and comparing these features to those of an apparently healthy control group; 2. Comparing the evolution of ECG alterations in men versus women with classical FD. And the secondary aim is: 3. Investigating the relationship between ECG features and left ventricular mass and the presence of late gadolinium enhancement on CMR in classical FD patients.
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