Maresi E, Albano N, Procaccianti P, Campesi G
Istituto di Anatomia ed Istologia Patologica, Università degli Studi di Palermo.
G Ital Cardiol. 1990 Jun;20(6):555-61.
In this study two autoptic cases of right ventricular dysplasia, observed in young and asymptomatic subjects who died suddenly are reported. In these patients, the "primary" myocardial atrophy involved the right ventricle, the right atrium and the conduction system: the sinoatrial node and the internodal pathways (in both cases) and the hisian bifurcation (only in the second case). The primary ventricular dysplasia was always associated with "secondary" transmural hyperplasia of the sub-epicardial fat tissue except for the anterior wall of the pulmonary infundibulum. According to the findings observed, we think that: 1) in all cases of right ventricular dysplasia a careful examination of the conduction system must be performed due to the fact that the lethal arrhythmias can be not only "hyperkinetic" but also "hypokinetic" arrhythmias; 2) right ventricular dysplasia is a dysplastic-congenital disease and transmural fatty hyperplasia depends both on "primary" myocardial atrophy and on the presence of sub-epicardial fat tissue; the latter is related to the age of the patient.
本研究报告了两例右心室发育异常的尸检病例,患者为年轻且无症状的猝死个体。在这些患者中,“原发性”心肌萎缩累及右心室、右心房及传导系统:窦房结和结间通路(两例均有)以及希氏束分叉(仅在第二例中有)。除肺动脉漏斗部前壁外,原发性心室发育异常总是伴有心外膜下脂肪组织的“继发性”透壁性增生。根据观察结果,我们认为:1)在所有右心室发育异常病例中,必须仔细检查传导系统,因为致死性心律失常不仅可能是“运动亢进性”的,也可能是“运动减退性”的;2)右心室发育异常是一种发育异常性先天性疾病,透壁性脂肪增生既取决于“原发性”心肌萎缩,也取决于心外膜下脂肪组织的存在;后者与患者年龄有关。