Ebstein anomaly

Case contributed by Vincent Tatco


Patient presented with chest pain and difficulty of breathing. Physical examination revealed a murmur.

Patient Data

Age: 50 years
Gender: Female



There is massive cardiomegaly with enlarged right atrium.

The patient had a 2D echocardiogram which revealed the following findings:

Atrialized right ventricle with normal systolic function. Slightly thickened ventricular walls. There is flattening of the interventricular septum indicative of pressure overload. Tethered and apically displaced septal leaflet of the tricuspid valve (Apical displacement indexed= 18 mm/m²). The anterior leaflet is also tethered with Sail-like sign. Non-coaptation of all the leaflets resulting in severe regurgitation. Tricuspid regurgitation Vmax = 3.5 m/s; Peak gradient = 48 mmHg. Findings are consistent with Ebstein Anomaly.

Small left ventricular size (left ventricular end-diastolic diameter indexed to body surface area = 2.1 cm/m²). Mild systolic dysfunction. Ejection fraction by visual estimation: 50-55%. Normal filling pressure.

Normal left atrial size. Area = 8 cm².

Interatrial septum appears to be intact by color Doppler.

Severely dilated right atrial size. Area = 105 cm². Dilated inferior vena cava with <50% collapsibility. Estimated right atrial pressure= > 15 mmHg

Thickened mitral valve leaflets without restriction of motion. Moderate regurgitation

Thickened aortic cusps without restriction of motion. No regurgitation

Normal pulmonic valve. Mild regurgitation. Right ventricular outflow tract VTI = 12 cm

Normal aortic root diameter

Dilated main pulmonary artery dimensions

No pericardial effusion

Moderately elevated pulmonary pressures

Case Discussion

This is a case of an adult patient with echocardiographically proven Ebstein anomaly.  

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