Short PR interval in the absence of preexcitation: a characteristic finding in a patient with Fabry disease.
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.
Cardiac Arrhythmia Service, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts 02114, USA.
Arash
A
Aryana
Michael A
MA
Fifer
Jeremy N
JN
Ruskin
Theofanie
T
Mela
D000328:Adult; D001145:Arrhythmias, Cardiac / Q000175:diagnosis*; D004562:Electrocardiography / Q000379:methods*; D000795:Fabry Disease / Q000175:diagnosis*; D005260:Female; D006801:Humans