Paroxysmal tachycardias proved fatal in a middle-aged man with type A Wolff-Parkinson-White syndrome. Efforts to control his arrhythmias included a surgical incision into the left atrium, based on discovery of early left ventricular activation during epicardial mapping. The incision did not alter any electrocardiographic or clinical feature; at later necropsy examination it was found that the incision had not cut a nearby left atrioventricular (A-V) connection. Serial section study of the entire A-V rings and septal junction of this heart also demonstrated a second unusual A-V connection, between the atrial septum and the region of the His bundle. This latter connection was anatomically eccentric to the normal organization of this region and may have caused an alteration in the local electrophysiological behavior. The left lateral A-V connection may have been of no electrophysiological significance since it was composed of ordinary working myocardial cells. These and other possible correlations are discussed in the context of the clinical features, numerous electrophysiological observations, and the meticulously determined anatomical findings.
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