Fontaine G, Frank R, Guiraudon G, Pavie A, Tereau Y, Chomette G, Grosgogeat Y
Arch Mal Coeur Vaiss. 1984 Aug;77(8):872-9.
Correlations between surface ECGs, epicardial mapping and histological data in 15 cases of arrhythmogenic right ventricular dysplasia (ARVD) provide information about the possible mechanism of intraventricular conduction defects in about one third of cases. Two cases in this series had complete right bundle branch block and 4 cases showed diffuse intraventricular conduction defects. The other cases had normal ECGs. The point of origin of the activation in 11 cases was situated in the left ventricle, even in 6 of the 9 cases with normal ECGs. The points of latest activation were located over the right ventricular free wall near the atrio-ventricular groove. However, in all cases but one, a normal right ventricular point of origin was observed, suggesting participation of the right bundle within a free wall showing delayed activation. This activation showed very irregular delayed propagation due to the zones of dysplasia. These results suggest that in ARVD, the mechanism of the conduction defects is not disease of the bundle branch itself but a distal block probably situated in the right ventricular wall. This hypothesis is supported by the histological appearances of the dysplastic zones.
15例致心律失常性右室发育不良(ARVD)患者的体表心电图、心外膜标测与组织学数据之间的相关性,为约三分之一病例的室内传导缺陷可能机制提供了信息。该系列中有2例出现完全性右束支传导阻滞,4例表现为弥漫性室内传导缺陷。其他病例心电图正常。11例患者激动的起源点位于左心室,甚至在9例心电图正常的患者中有6例也是如此。最晚激动点位于房室沟附近的右心室游离壁上。然而,除1例之外的所有病例中,均观察到正常的右心室起源点,提示右束支参与了激动延迟的游离壁。由于发育异常区域,这种激动显示出非常不规则的延迟传导。这些结果表明,在ARVD中,传导缺陷的机制不是束支本身的疾病,而是可能位于右心室壁的远端阻滞。发育异常区域的组织学表现支持这一假说。