James T N, MacLean W A
Chest. 1980 Jul;78(1):24-30. doi: 10.1378/chest.78.1.24.
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.
一名有晕厥发作史的高中运动员突然死亡。阵发性室性心律失常曾多次被记录，心室颤动至少发作过一次。一名有类似病史的兄弟也在大约相同年龄突然意外死亡。除了晕厥和心律失常外，他们两人都被认为身体健康。尸检时，未发现明显的心外异常，心脏大体检查正常。对心脏传导系统进行了专门研究。在心脏内的各个部位，包括房室结，发现了小神经和神经节的局灶性炎性变性，但在窦房结及其周围尤为明显。该区域有心外膜水肿和心包增厚，但其他部位的心包均正常。存在一些持续性的房室结胎儿期离散现象。文中讨论了这些神经病变可能导致阵发性心律失常发病机制并最终导致猝死的方式。还考虑了神经疾病的可能病因及其家族性发病的基础。
