Ebstein's anomaly: epidemiological analysis and presentation of different prenatal management

Abstract

Objectives

To present the crucial role of echocardiographic examination in perinatal care and analyze influence of prenatal treatment for neonatal outcome. Furthermore, the attempt to answer the question if there was any relationship between the occurrence of fetal Ebstein’s anomaly and environmental risk factors in polish population.

Methods

Forty-five prenatal diagnoses of Ebstein’s anomaly were compiled over the 21-year period (1998‒2018) form our single unit. The analysis included the assessment of maternal parameters (age, past obstetric history, and place of residence) and fetal parameters (sex, gestational age, anatomy, the fetal cardiovascular condition assessed by the CVPS, associated extracardiac anomalies or malformations, prenatal treatment, delivery and follow-up).

Results

The average age of gravida was 29.5 years (± 5.2 years) and gravidae <35 years of age accounted for 80% . There were 43 singleton pregnancies and 2 cases of multiple pregnancy. Ebstein’s anomaly was mostly (averagely) diagnosed at 28th week of gestation. Forty-three fetuses had normal karyotypes and two had trisomy 21. Cardiomegaly was present in 91% (41) of fetuses. The average heart area to chest area ratio was 0.56 (± 0.12). In 21 cases, there was only fetal monitoring – echocardiographic examinations and postnatal mortality was 44.4%. In 5 cases, transplacental digoxin treatment was administered and mortality was: 40%. In another 5 cases, only steroid therapy was applied and postnatal mortality was 100%. Steroids and transplacental digoxin treatment were administered in 11 cases and mortality was 63.6%. In 3 last cases transplacental digoxin treatment, steroids and maternal hyperoxygenation therapy were given and mortality was 0%. Cesarean section rate was 49%. Moreover, due to Ebstein’s anomaly regional peak of occurrence benzopyrene was deliberated as environmental risk factor.

Conclusions

Fetal Ebstein’s anomaly occurred in our population in healthy young women, expecting their first child and malformation was not related to fetus gender, nor to maternal health condition. Our data can be a new signal for the development of novel treatment strategies in therapy in fetuses with Ebstein’s anomaly.

Keywords:

• Prenatal treatment
• fetal echocardiography
• Ebstein’s anomaly

Acknowledgments

The authors would like to express special thanks for our colleagues and co-workers from the Polish Mother’s Memorial Hospital, who took part in the chain of departments taking care of pregnant woman and newborns with heart defects in our hospital:

Head of Obstetrics, Perinatology and Gynecology Department – Prof. M. Grzesiak

Head of Genetic Department – A. Gach, MD

Head of Neonatology Department – Prof. E. Gulczyńska

Head of Intensive Care and Congenital Defects Department – Prof. I. Maroszyńska

Head of Cardiology Department – Prof. Jadwiga Moll

Head of Cardiac Surgery Department – Prof. Jacek Moll

Additional special thanks for Izabela Pietrzyk, MA for her input in our database.

Disclosure statement

No potential conflict of interest was reported by the author(s).