DE SUBITANEIS MORTIBUS .30. OBSERVATIONS ON PATHOPHYSIOLOGY OF LONG QT SYNDROMES WITH SPECIAL REFERENCE TO NEUROPATHOLOGY OF HEART
DE SUBITANEIS MORTIBUS .30. OBSERVATIONS ON PATHOPHYSIOLOGY OF LONG QT SYNDROMES WITH SPECIAL REFERENCE TO NEUROPATHOLOGY OF HEART
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DOI：
10.1161/01.cir.57.6.1221
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发表时间：
1978-01-01
期刊：
影响因子：
37.8
通讯作者：
NORTH, RL
中科院分区：
文献类型：
--
作者：
JAMES, TN;FROGGATT, P;NORTH, RL
8例突触发作、心电图QT间期延长的患者(不同家系)突然死亡。5名听力正常，3名先天失聪。8例心脏尸检均为窦房结、房室结、希氏束和心室肌内的局灶性神经炎和神经变性。虽然这种心内神经疾病的病因尚不清楚，但慢性病毒感染或某些非感染性退行性病变都是可能的原因。如果心内神经炎和神经变性在其他死于长QT综合征的患者中被普遍发现，那么应该进一步考虑在发病机制中是否存在真正的遗传因素。由于心脏神经病变的不对称性和局灶性分布，对目前治疗长QT间期综合征的内科或外科治疗的反应可能因益处而异。在更多地了解神经疾病的真正病因之前，治疗可能仍将是经验性的，应该谨慎地进行临床观察。
Eight patients (different families) with syncopal attacks and a long QT interval in the ECG died suddenly. Five heard normally and 3 were born deaf. At postmortem examination of all 8 hearts the single consistent abnormality was focal neuritis and neural degeneration within the sinus node, A-V [atrioventricular] node, His bundle and ventricular myocardium. Although the etiology of this intracardiac neural disease is uncertain, a chronic viral infection or some noninfectious degenerative process are among the plausible causes discussed. If intracardiac neuritis and neural degeneration prove to be a prevalent finding among other victims dying from the long QT syndromes, further consideration should be given to whether there is any genuine hereditary component in the pathogenesis. Because of the asymmetrical and focal distribution of the cardioneural lesions, the response to present forms of medical or surgical treatment of the long QT syndromes may vary from benefit to harm. Until more is known of the true etiology of the neural disease, treatment will probably remain empirical in nature and should be conducted with cautious clinical observation.