PQ Interval in Patients With Fabry Disease

2010

Fabry disease (FD) is an X-chromosomal inherited lysosomal storage disease resulting in intracellular storage of globotriaosylceramide. Cardiac involvement is most frequently manifested as left ventricular hypertrophy. However, patients with FD may also have from various conduction abnormalities particularly affecting atrioventricular (AV) conduction. The present study was designed to analyze primarily AV conduction abnormalities on baseline electrocardiograms of patients with FD and to investigate the correlation with echocardiographic findings. Electrocardiograms at rest of 207 patients with FD were compared to echocardiograms. PQ-interval shortening and first-degree AV block could be fou…