Ebstein anomaly

Case contributed by David Cuevas


Low weight, known Wolff-Parkinson-White syndrome, holosystolic murmur along the lower left sternal border

Patient Data

Age: 15 years
Gender: Male

Ebstein's anomaly with 73% tethering, atrialization of 54% and Celermajer's index of 0.97 (grade II)(see annotated image).

Moderate tricuspid insufficiency, right atrial dilatation.

Systolic dysfunction of both ventricles (LVEF 35.29% and RVEF of 34.4%)

The right ventricle area is 44.8 cm2 and its atrialized portion is 24.4 cm2 with atrialization of 54%. The tricuspid leaflets are elongated and attached to the walls, the posterior one of 54 mm with a 73% attachment and the septal with a length of 55 mm and an adjoining of 74%.

The left ventricle, has a diastolic diameter of 40.5 mm, systolic diameter of 28 mm, septal wall thickness of 6 mm and posterior wall of 5 mm.

Annotated image

Celermajer severity index measures the area of the RA and atrialized RV (aRV) divided by the area of the functional RV(fRV), LA, and LV .

[RA+aRV]/ [fRV+LA+LV] from 4 chamber view.

It is graded from I to IV (grade I: index <0.5, grade II: 0.5–0.99, grade III: 1.0–1.49, grade IV >1.5). 

Also with a left ventricular false tendon.

Case Discussion

This is a typical case of Ebstein anomaly, a rare congenital malformation of the heart that is characterized by apical displacement of the septal and posterior tricuspid valve leaflets, with atrialization of the right ventricle due to the failure of delamination.

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