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A 'box-shaped' heart is a radiographic description given to the cardiac silhouette in some cases of Ebstein anomaly. The appearance of this finding is caused by the combination of the following features: huge right atrium that may fill the entire right hemithorax shelved appearance of the left cardiac contour due to the dilated right ventricular outflow tract or elevated residual right ventricle small aorta and main pulmonary artery.

Ebstein's Anomaly - Mayo Clinic Joseph Dearani, M.D., cardiovascular surgeon at Mayo Clinic

Ebstein's Anomaly. Split S2 and a systolic murmur can be heard.

Ebstein's Anomaly: cyanosis; a/w WPW. Tx procainamide or propafone (no BB, CCB !). Surgery if severe (decr exercise capacity, very tachycardic, cyanosis w/resting O2 sat <90%, symptomatic TR, TIA/CVA)

Ebstein's Anomaly atrial septal defect, atrialized right ventricle, malformed tricuspid valve Associated with lithium exposure in 1st trimester


Ebstein's malformation of the tricuspid valve is characterized by displacement of the septal and posterior leaflets of the tricuspid valve downward into the right ventricle (RV) to varying degrees. The portion of the RV proximal to the valve is called 'atrialized RV'. The anterior leaflet of the valve is usually normal but can be affected. The valve's annulus is normally located. The anatomy of the leaflets is variable and the chordal attachments are abnormal. -Yale School of Medicine

Congenital Heart Disease : Ebstein's Anomaly