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病态窦房结综合征(SSS)最主要特征为心动过缓。另一种虽也伴有双结功能异常,但主要特征为长P-R的病例则尚未见报导。本文描述两例同一家族的长P-R综合症病例。结合文献讨论了该病发病的电生理机制。提出其病因可能为一种先天性的心内迷走分支功能异常,或心内先天性胆硷酯酶缺乏,从而导致迷走活动后,乙酰胆硷释放与分解的动态平衡失调,使迷走功能经常处于紧张状态而发病。
Sick sinus syndrome (SSS) is the most important characteristic of bradycardia. The other, which is also associated with abnormal dual-node function, has not yet been reported for the main features of long P-R. This article describes two cases of long P-R syndrome in the same family. Combined with the literature discussed the pathogenesis of the electrophysiological mechanism. Proposed etiology may be a congenital intracardiac vagal dysfunction, or congenital cholereptase deficiency in the heart, resulting in vagal activity, acetylcholine release and decomposition of the dynamic balance of dysfunction, often in the vagal function at Tension and disease.