Fabry disease (FD) is a rare, inherited, slowly progressive X-linked lysosomal storage disorder. Mutations in the galactosidase alpha gene (GLA) are the primary cause of FD, leading to a decreased activity of the lysosomal enzyme alpha-galactosidase A (AGAL). This results in intracellular accumulation of the enzymes’ main substrate, globotriaosylceramide (Gb3) in various organs, including the heart, which ultimately lead to organ damage. During adulthood, symptomatic cardiac disease in the form of conduction abnormalities, arrhythmias, ischemic heart disease and heart failure may arise, ultimately leading to cardiac death in many patients. Alterations in electrophysiological markers and cardiac morphology and function precede the development of clinical heart disease. Currently, knowledge on the course of these changes and in which patients they arise is lacking. Specifically, the age of occurrence, the progression rate and how these markers of cardiac disease are linked to clinical outcomes is limited. The main goal of the thesis is to study the cardiac manifestations of FD, the course of electrophysiological and echocardiographic features in men and women with FD throughout adult life, and how they differ from the healthy population. With this knowledge, it will be possible to identify and treat FD patients at risk for developing cardiac events and to develop tailored follow-up protocols taking these prognostic markers into account.