Ebstein's anomaly: outcome of pregnancy

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.