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

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Ebstein's Anomaly. Associated c maternal use of lithium during pregnancy. Split S2 and a systolic murmur can be heard.

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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 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

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