[Congenital atrioventricular block and maternal lupus erythematosus. Histologic discovery of tumor of the atrioventricular node].

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.