Ebstein’s malformation in the fetus with cardiomegaly. To treat or to observe? – a case report

Abstract

Abstract Ebstein’s malformation is a rare congenital cardiac defect characterized by an abnormal formation and /or displacement of the leaflets of the tricuspid valve. Prognosis for a neonate is poor in case of cardiomegaly, due to coexistence of lung hypoplasia. This paper presents a case of a fetus with Ebstein’s malformation with massive cardiomegaly (HA/CA = 0.62) in a 27-year-old patient in her first pregnancy. The cardiac defect was diagnosed in the 22nd week of pregnancy. Due to the fact that the patient decided to continue her pregnancy, and taking into consideration single reports of transplacental Digoxin therapy, an attempt to apply Digoxin therapy was made. The mother and the fetus were monitored in two centers, in Rzeszów and in Łódź. In the course of a 12-week transplacental therapy, 8 fetal echocardiography examinations were performed and the following parameters improved: HA/CA (0.62-0.5), CVPS (5/10-7/10) SF RA (0%-11%), SF RV (18%-28%). There was also a conversion of the oxygen test from negative to positive, which seems to document that prevention of the lung hypoplasia was achieved. The neonate died on the 8th day of postnatal life before a cardiac surgery attempt.