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Ebstein anomaly is a rare congenital heart defect characterized by downward (apical) displacement of the septal and posterior tricuspid valve leaflets into the right ventricle, causing "atrialization" of the right ventricle and tricuspid regurgitation (TR).
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Ebstein’s anomaly describes a problem with a valve on the right side of your heart, the tricuspid valve.
Ebstein’s Anomaly: Causes, Symptoms & Treatment. Cleveland Clinic. Published March 16, 2017. Accessed July 11, 2025. https://my.clevelandclinic.org/health/diseases/16946-ebsteins-anomaly-for-adults
Key anatomical features:
| Feature | Description |
|---|---|
| Apical displacement | >8 mm/m² body surface area (BSA) between tricuspid and mitral valve insertion points |
| Atrialized RV | The portion of RV proximal to the displaced leaflets functions like part of the right atrium |
| Functional RV | Small and compressed distal portion of RV that still pumps blood to the pulmonary artery |
| Tricuspid regurgitation | Common due to leaflet tethering, redundancy, or dysplasia |
| Age | Symptoms |
|---|---|
| Neonates | Cyanosis, heart failure, poor perfusion |
| Infants/Children | Cyanosis, exercise intolerance, arrhythmias |
| Adolescents/Adults | Palpitations, exertional dyspnea, fatigue, paradoxical emboli (via ASD) |
Clinical manifestations and pathophysiological substrates of Ebstein’s anomaly. The interplay between arrhythmias and heart failure is highlighted. RA dilation promotes the occurrence of intra-atrial re-entries and the presence of accessory pathways may be the substrate for atrioventricular re-entries; aRV is vulnerable to trigger ventricular arrhythmias. Fast-conduced arrhythmias may contribute to ventricular dysfunction and reduced filling time. Adverse myocardial remodelling may enhance the arrhythmic substrate. Increased RA pressures favour right-to-left shunt in the presence of atrial communications, thus worsening hypoxia. Systemic embolisation is favoured by occurrence of atrial fibrillation or paradoxical thrombosis. Ao, aorta; ARV, anatomical right ventricle; aRV, atrialised right ventricle; LA, left atrium; L-R, left to right; LV, left ventricle; PA, pulmonary artery; R-L, right to left; RA, right atrium.
Pasqualin G, Boccellino A, Chessa M, et al. Ebstein’s anomaly in children and adults: multidisciplinary insights into imaging and therapy. Heart. 2023;110(4):235-244. doi:https://doi.org/10.1136/heartjnl-2023-322420
Physical examination: