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临床病理相关性。猝死。第二十五部分。结节病性心脏病。

Clinicopathologic correlations. De subitaneis mortibus. XXV. Sarcoid heart disease.

作者信息

James T N

出版信息

Circulation. 1977 Aug;56(2):320-6. doi: 10.1161/01.cir.56.2.320.

Abstract

Postmortem findings within the cardiac conduction system are described from the case of a black woman with sarcoid heart disease who died suddenly. Her clinical course had been characterized by recurring ventricular arrhythmias and bouts of syncope. Both the sinus node artery and the atrioventricular (A-V) node artery were sites of focal fibromuscular dysplasia, which thickened slightly the wall of the former but markedly narrowed the lumen of the latter. Small foci of sarcoid infiltration were present in the sinus node and the A-V node. Fatty replacement within the His bundle was attributable to the probable ischemia caused by narrowing of the A-V node artery. Sarcoid granulomata and infiltration with epithelioid cells were present throughout the ventricular myocardium, but were conspicuously less prevalent in the atria. All the large coronary arteries were normal. Many small coronary arteries in the ventricular myocardium were involved by the sarcoidosis and their lumen were narrowed. These findings and analogous ones reported by others are discussed relative to the pathogenesis of syncopal attacks and sudden death which seem to peculiarly prevalent in sarcoid heart disease.

摘要

本文描述了一名患有结节病性心脏病的黑人女性突然死亡病例的心脏传导系统尸检结果。她的临床病程特点为反复发作的室性心律失常和晕厥发作。窦房结动脉和房室结动脉均存在局灶性纤维肌发育异常,前者动脉壁轻度增厚,后者管腔显著狭窄。窦房结和房室结有小灶性结节病浸润。希氏束内的脂肪替代可能归因于房室结动脉狭窄导致的缺血。整个心室心肌均有结节病肉芽肿和上皮样细胞浸润,但心房中明显较少见。所有大冠状动脉均正常。心室心肌中的许多小冠状动脉受累于结节病,管腔狭窄。结合其他人报告的类似发现,对结节病性心脏病中似乎特别常见的晕厥发作和猝死的发病机制进行了讨论。

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