日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

Background: There are few reports that focus specifically on the prognostic factors for fetal cases of congenital complete atrioventricular block (CCAVB) with positive maternal anti-SS-A antibodies, and the impact of maternal symptoms and anti-SS-A antibody levels on fetal prognosis remains unclear. The aim of this study was to elucidate the risk factors for intrauterine fetal death (IUFD) in fetuses with CCAVB and positive maternal anti-SS-A antibodies.

Method: We retrospectively analyzed 47 fetal cases of CCAVB born to mothers with positive anti-SS-A antibodies at 66 hospitals in Japan from 1996 to 2010. Clinical characteristics and measurements between the IUFD group (n＝7) and the live-birth group (n＝40) were compared.

Results: Cases of fetal heart rate ＜55 beats/minute at diagnosis of CCAVB (57％ vs 17％, p＜0.05) and development of hydrops fetalis during follow up (71％ vs 20％, p＜0.05) were more in the IUFD group than the live-birth group. Advanced maternal age was also associated with IUFD. Multivariate analysis showed that hydrops fetalis and advanced maternal age were independent risk factors for IUFD. There were no significant differences between the groups in the frequency of mothers with symptoms of connective tissue diseases, maternal anti-SS-A antibody levels, and the rate of transplacental administration of steroids.

Conclusion: Hydrops fetalis and advanced maternal age are independent risk factors for IUFD. Fetuses with CCAVB and positive maternal SS-A antibodies should be closely followed up for hydrops fetalis so that they can be delivered in a timely fashion.