title: PQ interval in patients with Fabry disease.
abstract: 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.
authors.affiliation: Clinic for Cardiology, University Hospital of Zurich, Zurich, Switzerland. mehdi.namdar@usz.ch
authors.forename: Mehdi
authors.initials: M
authors.lastname: Namdar
authors.forename: Christoph
authors.initials: C
authors.lastname: Kampmann
authors.forename: Jan
authors.initials: J
authors.lastname: Steffel
authors.forename: Daniel
authors.initials: D
authors.lastname: Walder
authors.forename: Johannes
authors.initials: J
authors.lastname: Holzmeister
authors.forename: Thomas Felix
authors.initials: TF
authors.lastname: Lüscher
authors.forename: Rolf
authors.initials: R
authors.lastname: Jenni
authors.forename: Firat
authors.initials: F
authors.lastname: Duru
mesh_terms: D000328:Adult; D001145:Arrhythmias, Cardiac / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000503:physiopathology; D054537:Atrioventricular Block / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000503:physiopathology; D015331:Cohort Studies; D015150:Echocardiography, Doppler; D004562:Electrocardiography; D000795:Fabry Disease / Q000150:complications / Q000000981:diagnostic imaging* / Q000503:physiopathology*; D005260:Female; D006801:Humans; D017379:Hypertrophy, Left Ventricular / Q000150:complications / Q000000981:diagnostic imaging / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D055815:Young Adult