A 63 year old man with complete atrioventricular block was diagnosed as having Fabry's disease. A short PR interval is a common electrocardiographic finding in Fabry's disease, but complete atrioventricular block is a very rare complication. Necropsy indicated that lipid accumulation in the atrioventricular conduction system was the probable cause of this patient's atrioventricular block.
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