Pregnancy outcomes in women with Ebstein's anomaly: data from the EORP Registry of Pregnancy and Cardiac Disease (ROPAC)

Abstract

Abstract Background Ebstein's anomaly is a rare congenital cardiac condition characterized by displacement of the posterior and septal leaflets of the tricuspid valve towards the apex of the right ventricle. The ESC Guidelines for the management of cardiovascular diseases during pregnancy categorize women with uncomplicated Ebstein's anomaly as modified World Health Organization (mWHO) risk class II. However, data regarding pregnancy outcomes in women with Ebstein's anomaly are scarce. Purpose To evaluate the maternal and perinatal risks of pregnancy in women with Ebstein's anomaly. Methods All patients with Ebstein's anomaly who had been included in the Registry of Pregnancy and Cardiac Disease (ROPAC), which is an international, prospective, observational registry of pregnant women with underlying cardiac disease (n=5739), were evaluated. The primary outcome was the occurrence of a major adverse cardiac event, defined as maternal mortality, heart failure, arrhythmia, thromboembolic events or endocarditis. The secondary outcomes were obstetric and perinatal outcomes and the influence of pregnancy on tricuspid valve regurgitation. Results Thirty-six hospitals in 22 countries enrolled 81 women with Ebstein's anomaly (mean age 29.7 years, 46.9% nulliparous). Seven (8.6%) women had a history of tricuspid valve repair and a further eight (9.9%) of tricuspid replacement. Most women (67.9%) were in NYHA class I. At least one major adverse cardiac event occurred in 8 (9.9%) pregnancies, including heart failure (n=6), supraventricular arrhythmia (n=3) and thromboembolic events (n=2). There was no maternal death. Almost half of the women underwent a Caesarean section (49%) and preterm delivery occurred in 24.7%. Neonatal mortality was 2.5% and four (4.9%) infants had neonatal congenital heart disease. Serial echocardiographic data pre- and postpregnancy were available in 14 women. There was no clear deterioration in tricuspid regurgitation (see Picture 1). Conclusion Although mortality was zero, in 10% of the pregnant women with Ebstein's anomaly a major adverse cardiac event occurred, most frequently heart failure or arrhythmia. Therefore, women with Ebstein's anomaly should be categorized as mWHO risk class II at least, but perhaps more correctly as II–III. Preconception counseling is crucial, so that women are aware of the potential risks of maternal morbidity, preterm delivery and congenital heart disease and the need for careful monitoring during pregnancy. Funding Acknowledgement Type of funding sources: None.