Ebstein's Anomaly

What is Ebstein's anomaly?

Ebstein’s anomaly is an abnormality in the tricuspid valve (the heart valve that connects the right atrium with the right ventricle). This abnormality may cause the valve to leak, resulting in enlargement of the right atrium. Right atrial enlargement can lead to problems including arrhythmia and poor lung development. Ebstein’s anomaly occurs in one in 10,000 live births.

How is this condition managed during pregnancy?

When an Ebstein’s anomaly is suspected, further evaluation includes a specialized evaluation of the fetal heart (fetal echocardiogram). As it can be associated with other cardiac abnormalities and genetic syndromes, genetic counseling and testing may be recommended. Prenatal consultations with pediatric cardiology, pediatric cardiothoracic surgery, and neonatology specialists are recommended to discuss the prognosis and management after birth. Serial ultrasound and echocardiogram studies will be performed to monitor this condition over time. Prenatal care will be managed by a maternal-fetal medicine specialist, an obstetrician with special training and expertise in high-risk pregnancies.

How is this condition managed after delivery?

Newborns will be evaluated and cared for by a team involving pediatric cardiology and neonatology specialists. Outcomes vary widely based upon severity of the condition. Newborns with mild-to-moderate Ebstein’s anomaly have generally good results. However, severe disease has a guarded prognosis, with a high rate of newborns passing away within the first year of life.