Ebstein anomaly

Ebstein's anomaly is a very rare congenital heart defect that is associated with a malformed tricuspid valve and an often very thin-walled right ventricle.  The tricuspid valve is the ‘valve’ between the right atrium (RA) and the right ventricle (RV), which prevents the backflow of blood. The pumping capacity of the thin-walled ventricle is often restricted by the malpositioned tricuspid valve. The leaky tricuspid valve causes a back and forth flow between the right atrium and the right ventricle.

If the disease is mild, those affected usually do not notice it. If Ebstein's anomaly is severe, they may suffer from the following symptoms:

• Irregular and unusually strong heartbeats
• shortness of breath
• Difficulty drinking
• oedema
• Swelling of the liver
• Accumulation of fluid in the abdominal cavity
• Bluish discolouration of the skin
• Cardiac arrhythmia

For diagnostics, our doctors first use echocardiography and an electrocardiogram (ECG) to determine the right heart strain. They also often carry out an additional cardiac catheterisation.

The need for surgery will depend on the severity of the leak in the tricuspid valve and the restriction of the pumping function of the right ventricle. If an atrial septal defect (ASD) is often also present, cyanosis and cardiac arrhythmia or cardiac insufficiency and embolisms may make surgery necessary. Surgical treatment may therefore be necessary in the neonatal period or only in adulthood.