Ebstein's anomaly in pregnancy: maternal and neonatal outcomes

Ebstein's anomaly is a rare congenital cardiac abnormality, associated with cyanosis and arrhythmia. Patients often reach childbearing age and pregnant women pose a challenge to the treating physician. We describe the course and outcome of pregnancy in women with Ebstein's anomaly and discuss the related management issues.

Analysis of pregnancies in four women with Ebstein's anomaly was carried out in a referral institute in northern India. Data of two women were analyzed retrospectively and the other two women were prospectively followed in their pregnancies during the study period. The course of the pregnancy, disease and perinatal outcome in each woman were analyzed.

Four women had eight pregnancies all resulting in vaginal deliveries. There were two premature deliveries. The mean birth weight was 2.54 + or - 0.88 kg. Of the eight babies, six did not have any cardiac anomalies; one was an unexplained neonatal death and for another, no data was available. Pregnancy was well tolerated in two patients, while one had right heart failure during early pregnancy, and one had arrhythmia during labor, which was managed medically; one patient had arrhythmia in the postpartum period, which was managed medically.

When a woman with Ebstein's anomaly reaches childbearing age, fertility is not affected, even in cyanotic women. Under close supervision by the woman's obstetrician and cardiologist, the pregnancy outcome is usually favorable.