James T N, Marilley R J, Marriott H J
Circulation. 1975 Apr;51(4):743-8. doi: 10.1161/01.cir.51.4.743.
A seventeen-year-old girl in apparent good health was found dead in bed one morning. Two clinical features of note were multiple premature beats present in her electrocardiograms for several years, and a tall thin habitus without stigmata of Marfan's syndrome. At necropsy examination the heart was grossly normal. At its margin abutting the central fibrous body the atrioventricular (A-V) node exhibited numerous fronds and outcroppings, some forming loop connections from one part of A-V node to another. Groups of A-V nodal cells detached from the A-V node were also found connected to the crest of the interventricular septum. There were large connections directly from the His bundle to the crest of the interventricular septum (Mahaim fibers). The A-V node artery was moderately narrowed but patent. The possible significance of these findings in the pathogenesis of re-entrant arrhythmias or ectopic automaticity is discussed.
一名看似健康的17岁女孩某天早晨被发现死在床上。值得注意的两个临床特征是，她的心电图显示多年来存在多次早搏，以及身材瘦高但无马凡综合征体征。尸检时心脏外观正常。在靠近中心纤维体的心脏边缘，房室（A-V）结呈现出许多叶状结构和突出物，一些形成了从房室结一部分到另一部分的环状连接。还发现从房室结分离出的几组房室结细胞与室间隔嵴相连。有直接从希氏束到室间隔嵴的大连接（Mahaim纤维）。房室结动脉中度狭窄但通畅。讨论了这些发现对折返性心律失常或异位自律性发病机制的可能意义。
