114 Chapter 4 disease [8, 14-17]. Previous studies have shown that some morphological (Left Ventricular Mass) and functional (ratio of early diastolic mitral inflow velocity/ early diastolic septal tissue mitral annulus velocity (E/e’) and GLS) echocardiographic abnormalities predict the occurrence of cardiac complications in FD patients [18-23]. However, long-term longitudinal studies reporting progression of echocardiographic parameters in men and women with different FD phenotypes, and how this differs from healthy individuals, are lacking [18]. Comparing these echocardiographic features in FD patients with those of a healthy population, helps to recognize echocardiographic abnormalities that are typical for FD at both an early disease stage and later on in the disease course. Once we know how these features change over time and how they are related to known cardiac disease markers (e.g. plasma N-terminal prohormone of Brain Natriuretic Peptide (NT-proBNP) and troponin T) and the development of cardiac complications, these features can be used for decision making regarding treatment initiation and evaluation of therapeutic effectiveness. To establish the longitudinal echocardiographic changes in FD, we conducted a retrospective study in the FD patient cohort under follow-up at the Amsterdam University Medical Centre (AUMC) and compared the findings to those of a large healthy population study. The size of the FD patient cohort, length of follow-up and systematic review of all echocardiograms make this study unique, providing precise information on echocardiographic changes over time in FD patients. Aims of the study are: Primary: 1. Describing the echocardiographic features and their changes over time in men and women with classical FD and comparing these features to those of a healthy control group; 2. Comparing the changes in echocardiographic features between men and women with classical FD. Secondary: 3. Investigating the relation between echocardiographic features in FD patients and plasma NT- proBNP and Troponin-T levels; 4. Exploring the association between echocardiographic parameters and the subsequent development of cardiac complications.
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