Connolly H M, Warnes C A
Division of Cardiovascular Diseases and Internal Medicine, Mayo Clinic, Rochester, Minnesota 55905.
J Am Coll Cardiol. 1994 Apr;23(5):1194-8. doi: 10.1016/0735-1097(94)90610-6.
The outcome of pregnancy in Ebstein's anomaly was studied in 72 such patients (44 women, 28 men) who had had pregnancies or fathered children.
Patients with Ebstein's anomaly often reach childbearing age. Reports of the outcome of pregnancy in Ebstein's anomaly are available; however, the number of patients is small.
The medical and surgical data bases at the Mayo Clinic were reviewed, and 145 patients (62 men, 83 women) of childbearing age with Ebstein's anomaly were located. All patients were contacted, and 72 patients (44 women, 28 men) with offspring were identified and reviewed in detail to assess the outcome of pregnancy.
Forty-four women had 111 pregnancies resulting in 85 live births (76%). Seventy-six deliveries (89%) were vaginal, and nine (11%) were by cesarean section. Twenty-three deliveries were premature. There were 19 spontaneously unsuccessful pregnancies, 7 therapeutic abortions and 2 early neonatal deaths. The mean birth weight of the infants born to cyanotic women was 2.53 kg, which was significantly lower than the mean birth weight of infants born to acyanotic women (3.14 kg [p < 0.001]). The overall incidence of congenital heart disease in the 158 offspring of parents with Ebstein's anomaly was 4% (6 of 158). The incidence of congenital heart disease was 6% (5 of 83) in the offspring of women with Ebstein's anomaly and 1% (1 of 75) in that of men. There was a 0.6% (1 of 158) incidence of familial Ebstein's anomaly. There were no serious pregnancy-related maternal complications, which included maternal death, stroke, congestive heart failure, arrhythmias or endocarditis.
Pregnancy in women with Ebstein's anomaly is well tolerated. It is associated with an increased risk of prematurity, fetal loss and congenital heart disease in the offspring. In addition, a significantly lower birth weight is found in the offspring of cyanotic versus acyanotic women with Ebstein's anomaly. Paternal Ebstein's anomaly also seems to result in an increased risk of congenital heart disease in the offspring compared with the incidence in the general population.
对72例患有埃布斯坦畸形且有妊娠史或育有子女的患者（44名女性，28名男性）的妊娠结局进行研究。
埃布斯坦畸形患者常达生育年龄。有关于埃布斯坦畸形妊娠结局的报道；然而，患者数量较少。
回顾梅奥诊所的医疗和手术数据库，找出145例生育年龄的埃布斯坦畸形患者（62名男性，83名女性）。联系了所有患者，确定并详细审查了72例有后代的患者（44名女性，28名男性）以评估妊娠结局。
44名女性有111次妊娠，其中85例活产（76%）。76例分娩（89%）为阴道分娩，9例（11%）为剖宫产。23例分娩早产。有19次自然妊娠失败、7次治疗性流产和2例早期新生儿死亡。发绀型女性所生婴儿的平均出生体重为2.53kg，显著低于非发绀型女性所生婴儿的平均出生体重（3.14kg [p<0.001]）。埃布斯坦畸形患者后代中先天性心脏病的总体发病率为4%（158例中有6例）。埃布斯坦畸形女性后代中先天性心脏病的发病率为6%（83例中有5例），男性后代中为1%（75例中有1例）。家族性埃布斯坦畸形的发病率为0.6%（158例中有1例）。无严重的妊娠相关母体并发症，包括母体死亡、中风、充血性心力衰竭、心律失常或心内膜炎。
埃布斯坦畸形女性的妊娠耐受性良好。其与后代早产、胎儿丢失和先天性心脏病风险增加有关。此外，与非发绀型埃布斯坦畸形女性相比，发绀型女性后代的出生体重显著更低。与一般人群的发病率相比，父源性埃布斯坦畸形似乎也会导致后代先天性心脏病风险增加。
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