[Intra-uterine detection of atrio-ventricular block in two children whose mother had Sjögren's syndrome]

A woman with isolated juvenile Sjögren's syndrome gave birth, at 3 years' interval, to two children with complete atrio-ventricular heart block (AVB). This is the first published case of AVB in children of mothers with Sjögren's syndrome without any clinical and laboratory evidence of connective tissue disease, notably lupus. Ultrasonography showed that the AVB was acquired in utero and occurred during the 23rd week of gestation. In both children the AVB was isolated, without any symptom of congenital malformation of the heart; there were no abnormalities of conduction in the mother. Early corticosteroid treatment of the mother's disease had no beneficial effect on AVB in the foetuses. Attempts to reproduce the condition experimentally met with failure.