Abnormalities seen in the four-chamber view

Ebstein’s anomaly is characterised by a variable downward displacement of the attachment of the tricuspid valve into the inflow portion of the right ventricle. In the four-chamber view, there is more pronounced off-setting of the tricuspid valve than normal. Colour or pulsed-wave Doppler may show tricuspid regurgitation, depending on the severity of the lesion. If there is a significant tricuspid regurgitation, there will be dilatation of the right atrium. Significant cardiomegaly may cause secondary lung compression and consequent hypoplasia as well as fetal hydrops and subsequent death. Pulmonary stenosis or functional atresia is often seen as concomitant lesions.

Prevalence

Ebstein’s anomaly is found in about 1 in 20,000 live births.

Other abnormalities

Ebstein’s anomaly is not commonly associated with extracardiac defects or chromosomal abnormalities.

Prognosis

Prognosis depends on the severity of the malformation. Severe cases may result in intrauterine death. On the other hand, mild or moderate forms of Ebstein’s anomaly can be asymptomatic and surgical treatment may not be necessary.

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