Zanardi F, Occari G, Cavazzini L, Grandi E, Tomasi A M
G Ital Cardiol. 1986 Jan;16(1):4-14.
A family with "arrhythmogenic right ventricular dysplasia (ARVD)" is described. ARVD is pathologically characterised by a partial or total degeneration of the right ventricular myocardium, replaced by fatty and fibrous tissue. This causes dangerous ventricular arrhythmias or congestive heart failure in infancy. About the three described patients, a sixteen-year-old subject died suddenly, and his anatomical and histopathological reports have been presented. Familiarity, rarely treated in literature, and the presence, in the died subject, of a damage of the left ventricle too, are the peculiar characteristic of these "ARVD" cases. The importance about instrumental non-invasive studies has been underlined in order to know early "ARVD", which can be deceptive clinically. The authors suppose there is surely, at the beginning of "dysplasia", a genetic alteration of the right cardiac musculature, but acquired noxae could insert afterwards, on this genetic basis.
本文描述了一个患有“致心律失常性右室发育不良（ARVD）”的家庭。ARVD的病理特征是右心室心肌部分或全部退化，被脂肪和纤维组织取代。这会在婴儿期引发危险的室性心律失常或充血性心力衰竭。关于所描述的三名患者，一名16岁的受试者突然死亡，并且已经展示了其解剖学和组织病理学报告。这些“ARVD”病例的独特特征是家族性（在文献中很少有治疗相关报道）以及死亡受试者中还存在左心室损伤。强调了仪器非侵入性研究对于早期发现“ARVD”的重要性，因为它在临床上可能具有欺骗性。作者推测在“发育不良”开始时，右心肌肉肯定存在基因改变，但在此基因基础上，后天的有害因素随后可能会介入。
