Paroxysmal ventricular arrhythmias and familial sudden death associated with neural lesions in the heart.

A high school athlete with history of syncopal attacks died suddenly. Paroxysmal ventricular arrhythmias had been documented many times, as had at least one episode of ventricular fibrillation. A brother with similar history also had died suddenly and unexpectedly at about the same age. Except for the syncope and arrhythmias, they were both considered to be in good health. At postmortem examination, no significant extracardiac abnormalities were found, and the heart was normal on gross examination. The cardiac conduction system was the subject of special study. Focal inflammatory degeneration of small nerves and ganglia was found in various sites within the heart, including atrioventricular node, but were especially prominent in and around the sinus node. There was epicardial edema and thickening of the pericardium in that vicinity, but all of the pericardium elsewhere was normal. Some persistent fetal dispersion of the atrioventricular node was present. Ways are discussed in which these neural lesions may have contributed to the pathogenesis of paroxysmal arrhythmias and eventually sudden death. The possible etiology of the neural disease and the basis for its familial occurrence are considered.