Ebstein’s anomaly

– The R valve leaflets are attached to the walls and septum of the right ventricle. There is subsequent ‘atrialization’ of a portion of the morphologic right ventricle (which is then contiguous with the right atrium). This causes the right atrium to be large and the anatomic right ventricle to be small in size.

S3 heart sound
S4 heart sound
Systolic murmur of tricuspid regurgitation
Middiastolic murmur along the lower left sternal border
Right atrial hypertrophy
Right ventricular conduction defects
Wolff-Parkinson-White syndrome often accompanies (50% HAVE)

-Risk factors: taking lithium, and in those with Wolff-Parkinson-White syndrome

Ebstein’s cardiophysiology typically presents as an (antidromic) AV reentrant tachycardia with associated pre-excitation. In this setting, the preferred pharmacological treatment agent is procainamide. Since AV-blockade may promote conduction over the accessory pathway, drugs like beta blockers, calcium channel blockers and digoxin are contraindicated.

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