Two cases of congenital complete atrioventricular block

SUGANUMA Izumi, IWASA Koichi, FUJISAWA Hidetoshi, YAMANAKA Kaoruko, MATSUSHIMA Hiroshi, YASUO Tadahiro, OKUBO Tomoharu, IWASAKU Kazuhiro, KITAWAKI Jo

doi:10.11437/sanpunosinpo.63.81

Most cases of advanced fetal bradyarrhythmia are caused by CCAVB (congenital complete atrioventricular block). CCAVB is quite rare and only occurs in one of 20,000 pregnancies. CCAVB is usually complicated by cardiac anomalies, and half of the cases demonstrating a normal fetal heart structure are complicated with maternal collagen diseases. CCAVB develops from 18 weeks gestation when maternal autoantibodies can pass through the placenta and injure the electrical conduction system of fetal heart. Children diagnosed as having CCAVB tend to be associated with postnatal cardiomyopathy. Although maternal therapy with β-stimulator and steroid has been conducted, the standard treatment has not yet been established. We present two cases of CCAVB diagnosed prenatally from mothers with anti-SS-A antibody. Case 1; CCAVB was found at 22 weeks gestation. We conducted dexamethasone therapy combined with ritodrine hydrochloride. Nevertheless, emergent caesarean section was performed at 31 weeks gestation due to a progression of fetal heart failure. Dexamethasone was not effective for improving fetal heart status. There were no apparent side effects of dexamethasone in the fetus, but the mother developed a moon face. Case 2; CCAVB was detected at 25 weeks gestation. We treated this case with prednisolone. Because there were no signs of fetal heart failure during the course of pregnancy, an elective caesarean section was scheduled at 38 weeks gestation. There were no apparent side effects of prednisolone therapy on either the mother or the fetus. In both cases, the babies have progressed well postnatally and cardiomyopathy has not been detected to date. Although the use of steroid hormones as a treatment for CCAVB complicated with maternal collagen diseases remains controversial, we conducted steroid hormone therapy to prevent neonatal cardiomyopathy that could be caused by placental transfer of maternal autoantibodies. Although prednisolone generally shows a lower rate of placental transfer than dexamethasone and its efficacy for the treatment of CCAVB remains unknown, there were no serious side effects in either the mother or baby and both prenatal heart failure and postnatal cardiomyopathy were apparently prevented in our case. The accumulation of further reports on the treatment of CCAVB including the selection of steroid hormones is needed. [Adv Obstet Gynecol, 63 (2) : 81-87, 2011 (H23.5)]

Two cases of congenital complete atrioventricular block

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