Ebstein’s anomaly is a congenital heart condition where the tricuspid valve is set lower in the right side of the heart (towards the apex), causing a bigger right atrium and a smaller right ventricle. The tricuspid valve does not function normally, resulting in tricuspid regurgitation (blood flowing back from the right ventricle to the right atrium). There is reduced right ventricular function.
Ebstein’s anomaly is often associated with an atrial septal defect with a right-to-left shunt. Blood flow from the right atrium to the left atrium allows blood to bypass the lungs, leading to cyanosis.
It is also often associated with Wolff-Parkinson-White syndrome and supraventricular tachycardia.
Presentation
Ebstein’s anomaly may be asymptomatic until adolescence or adulthood.
It may present with:
- Arrhythmias (e.g., supraventricular tachycardia)
- Heart failure (e.g., shortness of breath and oedema)
- Collapse or cardiac arrest
- Cyanosis (when there is an associated atrial septal defect)
Management
An echocardiogram confirms the diagnosis.
Medical management involves treating arrhythmias and heart failure.
Surgical management may involve tricuspid valve repair or replacement.
Last updated January 2025
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