Fabry's disease with complete atrioventricular block: histological evidence of involvement of the conduction system

A 63 year old man with complete atrioventricular block was diagnosed as having Fabry9s disease. A short PR interval is a common electrocardiographic finding in Fabry9s 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 patient9s atrioventricular block.