The attempt of pregnancy modification in a case of fetal Ebstein’s anomaly

Abstract

Introduction: Ebstein’s anomaly is a rare congenital heart defect (< 1% of all cases of congenital heart disease). Its distinctive feature is the displacement of the septal and posterior leaflets of the tricuspid valve towards the apex. The right atrium is enlarged, and the right ventricle is divided into two parts. The leaflets are often dysplastic. This leads to tricuspid regurgitation. Pulmonary stenosis or atresia is the most common associated defect. All these changes can lead, during the fetal life, to cardiomegaly, lung hypoplasia, heart failure of the fetus, or even intrauterine demise. Case presentation: A 39-year-old G3P3 at 23 weeks of gestation had a detection of heart disease. She was admitted to our Institute for further management. The prenatal echocardiography exam showed the fetus with Ebstein’s anomaly, pulmonary valve atresia, and cardiomegaly. Intrauterine digoxin and steroid treatment was started. Subsequent fetal echocardiographic monitoring was performed late in the third trimester. At 39 weeks of gestation the oxygen test was positive and oxygen therapy was ordered. Elective caesarean section was performed at 40 weeks of gestation, and a male newborn weighting 2860 g was delivered. On the 11th day of life, he underwent cardiac surgery in the same hospital and was discharged four weeks later in a good general condition. Conclusion: Despite the fact that there are no recommendations for intrauterine therapy for fetal Ebstein’s disease, this case shows the possibility of finding an alternative method of fetal treatment, which can be a combination of digoxin, steroids, and maternal oxygen therapy and early neonatal, handmade, tricuspid valve replacement.