Bharati S, Moskowitz W B, Scheinman M, Estes N A, Lev M
Congenital Heart and Conduction System Center, Christ Hospital and Medical Center, Oak Lawn, Illinois 60463.
J Am Coll Cardiol. 1991 Jul;18(1):179-86. doi: 10.1016/s0735-1097(10)80238-4.
The conduction system was studied by serial section in three patients with intractable supraventricular tachycardias originating from the atrioventricular (AV) junction who died suddenly. The three patients were a 6 month old girl (Case 1), a 5 month old boy (Case 2) and a 22 year old woman (Case 3). The latter had a pacemaker inserted after surgical ablation of the AV node. The heart was hypertrophied and enlarged in all. In Case 1, the AV node was partly within the central fibrous body and there was a left-sided AV bundle with acute necrosis in the summit of the ventricular septum, adjacent to the AV node and bundle. In Case 2, the coronary sinus was displaced cranially close to the central fibrous body, resulting in abnormality of the latter, with entrapment, distortion and division of the AV node and bundle into two distinct components within the central fibrous body. In Case 3, a left-sided AV node was connected to the atrial septum. The right AV node was completely interrupted by sutures and the penetrating and branching bundle and bundle branches were markedly fibrosed. In addition, the atrial septum and summit of the ventricular septum showed marked inflammatory reaction with fibrosis, which was more marked on the right ventricular side. Histologic examination of the conduction system in all three cases demonstrated congenital abnormalities of the AV junction that may be related to the tachycardia. These findings emphasize the need to carefully evaluate the atrial septum and AV junctional area, including the coronary sinus, before ablative procedures are undertaken.(ABSTRACT TRUNCATED AT 250 WORDS)
对3例因房室交界区起源的顽固性室上性心动过速而猝死的患者进行了连续切片研究,以观察其传导系统。这3例患者分别为1名6个月大的女孩(病例1)、1名5个月大的男孩(病例2)和1名22岁的女性(病例3)。后者在房室结手术消融后植入了起搏器。所有患者的心脏均有肥厚和增大。病例1中,房室结部分位于中心纤维体内,在室间隔顶部、紧邻房室结和房室束处有左侧房室束急性坏死。病例2中,冠状窦向上移位,靠近中心纤维体,导致中心纤维体异常,房室结和房室束在中心纤维体内被包裹、扭曲并分为两个不同的部分。病例3中,左侧房室结与房间隔相连。右侧房室结被缝线完全中断,穿入和分支束以及束支明显纤维化。此外,房间隔和室间隔顶部显示出明显的炎症反应伴纤维化,在右心室侧更为明显。对所有3例病例的传导系统进行组织学检查发现,房室交界区存在先天性异常,可能与心动过速有关。这些发现强调了在进行消融手术前,需要仔细评估包括冠状窦在内的房间隔和房室交界区。(摘要截取自250字)