Accelerated Atrioventricular Conduction in Fabry's Disease: A Case Report

Three unrelated men with Fabry's disease (angiokeratoma corporis diffu sum universale) and accelerated atrioventricular conduction are described. AQn His bundle electrogram in one patient who reported episodes of tachycardia demonstrated a prolonged deflection preceding the ventricular complex. This deflection is thought to represent an abnormal His bundle potential, possible resulting from glycolpid deposition. In the second patient the PR interval decreased from 0.18 to 0.11 second during 10 years, and in the third patient it decreased from 0.12 to 0.10 seconds during 13 years. Accelerated atrioventricu lar conduction may develop in Fabry's disease.