PQ interval in patients with Fabry disease

Mehdi Namdar, Christoph Kampmann, Jan Steffel, Daniel Walder, Johannes Holzmeister, Thomas Felix Lüscher, Rolf Jenni, Firat Duru
American Journal of Cardiology 2010 March 1, 105 (5): 753-6
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 found in only 29 cases (14%) and 3 cases (1.4%), respectively. No echocardiographic differences could be found in patients with and without PQ-interval shortening, including left ventricular hypertrophy, atrial size, and diastolic parameters. Furthermore, no correlation of the PQ interval with any echocardiographic parameters was detected. There was no difference between men and women in baseline clinical and electrocardiographic parameters. In conclusion, shortening of the PQ interval was not a common electrocardiographic finding in patients newly diagnosed with FD. Furthermore, no correlation with typical echocardiographic findings or disease stage in FD at baseline could be found.

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