Fontaliran F, Guillois B, Colin A, Blanc J J, Chabaud J J, Boog G, Rossi L, Guérot C
Service de cardiologie, hôpital Boucicaut, Paris.
Arch Mal Coeur Vaiss. 1989 Apr;82(4):609-13.
A case of congenital atrioventricular block in a newborn whose mother presented with systemic lupus erythematosus (SLE) is reported. Despite intensive care the child died a few hours after birth. Serial sections of the heart could be examined. Histology provided information on the appearance and distribution of the lesions. In particular, the sinus node was small for the child's age, and its supplying artery was found to have a hyperplastic media with adventitial sclerosis; the interatrial and interventricular septa showed subendocardial fibrosis invading the adjacent myocardium. Owing to the scarcity of systematic histopathological examinations, such lesions have seldom been described. In addition, a tumour of the atrioventricular node, known as mesothelioma or hemolymphangioma, was discovered. This case is exceptional in that histopathological findings similar to those described in SLE, though rarely as numerous, were associated with a very rare tumour never hitherto described in such a young patient. The relationship between the two categories of lesions is discussed.
报告了一例新生儿先天性房室传导阻滞病例，其母亲患有系统性红斑狼疮（SLE）。尽管进行了重症监护，该患儿仍在出生后数小时死亡。对心脏进行了连续切片检查。组织学提供了有关病变外观和分布的信息。特别是，窦房结相对于患儿年龄较小，其供应动脉的中膜增生并伴有外膜硬化；房间隔和室间隔显示心内膜下纤维化侵入相邻心肌。由于系统性组织病理学检查较少，此类病变很少被描述。此外，还发现了一种房室结肿瘤，称为间皮瘤或血管淋巴管瘤。该病例具有特殊性，因为尽管与SLE中描述的组织病理学发现相似的情况很少见，但在如此年轻的患者中却与一种从未在此类患者中描述过的非常罕见的肿瘤相关。讨论了这两类病变之间的关系。
