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Short PR Interval in the Absence of Preexcitation: A Characteristic Finding in a Patient with Fabry Disease
Author(s) -
ARYANA ARASH,
FIFER MICHAEL A.,
RUSKIN JEREMY N.,
MELA THEOFANIE
Publication year - 2008
Publication title -
pacing and clinical electrophysiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.686
H-Index - 101
eISSN - 1540-8159
pISSN - 0147-8389
DOI - 10.1111/j.1540-8159.2008.01088.x
Subject(s) - medicine , fabry disease , pr interval , cardiology , electrical conduction system of the heart , disease , permanent pacemaker , electrocardiography , fabry's disease , atrioventricular node , tachycardia , heart rate , blood pressure
Fabry disease (FD) is a rare lysosomal storage disease characterized by intracellular deposition of glycosphingolipids that can involve various organs including the heart. Here, we report the finding of a short PR interval in a patient with FD and recurrent syncope associated with short atrial‐His (A‐H) and His‐ventricular (H‐V) intervals in the absence of preexcitation. While electrocardiographic abnormalities are not uncommon among FD patients, there have been few reports on electrophysiological studies performed on these patients. The exact mechanism for the short A‐H and H‐V intervals remains unclear, but they may result from accelerated conduction in the AV node—His—Purkinje system.