Pregnancy outcomes in women with Ebstein's anomaly: Data from the Registry of Pregnancy and Cardiac disease (ROPAC)

Johanna A. Van Der Zande, Oktay Tutarel, Karishma P. Ramlakhan, Annemien E. Van Der Bosch, Roberto Bordese, Elvin Zengin, William E. Wagner, Lidia De Sousa, Piers Clifford, Mark R. Johnson, Roger Hall, Jolien W. Roos-Hesselink*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

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Abstract

Objective:

Ebstein's anomaly is a rare congenital cardiac condition and data regarding pregnancy outcomes in this patient group are scarce. We evaluated the maternal and perinatal risks of pregnancy in 81 women with Ebstein's anomaly. 

Methods:

The Registry of Pregnancy and Cardiac disease is a prospective global registry of pregnancies in women with structural cardiac disease. Pregnancy outcomes in women with Ebstein's anomaly were examined. The primary outcome was the occurrence of a major adverse cardiac event (MACE) defined as maternal mortality, heart failure, arrhythmia, thromboembolic event or endocarditis. Secondary endpoints were obstetric and perinatal outcomes and the influence of pregnancy on tricuspid valve regurgitation as well as right atrial and ventricular dimensions. 

Results:

In the 81 women with Ebstein's anomaly (mean age 29.7±6.1 years, 46.9% nulliparous), MACE occurred in 8 (9.9%) pregnancies, mostly heart failure (n=6). There were no maternal deaths. Prepregnancy signs of heart failure were predictive for MACE. Almost half of the women were delivered by caesarean section (45.7%) and preterm delivery occurred in 24.7%. Neonatal mortality was 2.5% and 4.9% of the infants had congenital heart disease. In the subgroup in which prepregnancy and postpregnancy data were available, there was no difference in tricuspid valve regurgitation grade or right atrial and ventricular dimensions before and after pregnancy. 

Conclusions:

Most women with Ebstein's anomaly tolerate pregnancy well, but women with prepregnancy signs of heart failure are at higher risk for MACE during pregnancy and should be counselled accordingly.

Original languageEnglish
Article numbere002406
JournalOpen Heart
Volume10
Issue number2
DOIs
Publication statusPublished - 7 Aug 2023

Bibliographical note

Funding Information:
This work was supported by the ESC EORP. Funding from 'Zabawas Foundation' and 'De Hoop Foundation' in addition to the support from EORP is greatly acknowledged. Since the start of EORP, the following companies have supported the programme: Abbott Vascular Int. (2011–2021); Amgen Cardiovascular (2009–2018); AstraZeneca (2014–2021); Bayer AG (2009–2018); Boehringer Ingelheim (2009–2019); Boston Scientific (2009–2012); The Bristol Myers Squibb and Pfizer Alliance (2011–2019); Daiichi Sankyo Europe (2011–2020); The Alliance Daiichi Sankyo Europe and Eli Lilly and Company (2014–2017); Edwards (2016–2019); Gedeon Richter Plc. (2014–2016); Menarini Int. Op. (2009–2012); MSD-Merck & Co. (2011–2014); Novartis Pharma AG (2014–2020); ResMed (2014–2016); Sanofi (2009–2011); Servier (2009–2021) and Vifor (2019–2022).

Publisher Copyright:
© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.

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