Electrocardiographic changes in early recognition of Fabry disease

BackgroundFabry disease (FD) is an inherited X-chromosomal lysosomal storage disease resulting in intracellular storage of globotriaosylceramide. Cardiac involvement is most frequently manifested as left ventricular hypertrophy (LVH). However, patients with FD may also have various conduction abnormalities before LVH develops. The present study was designed to analyse early conduction abnormalities on baseline ECG of patients with FD and to investigate their diagnostic value.Methods and resultsBaseline electrocardiographic (ECG) and echocardiographic measurements of patients with FD (n=30) were compared with those of heart rate and age-matched healthy individuals (n=50). The PQ-interval (131±18 vs 155±20 ms, p