Ebstein's Anomaly

Abstract

Findings have been correlated with those reported in the literature to emphasize certain clinical features that are of diagnostic value. The characteristic ecg has tall P waves, frequently prolonged atrioventricular conduction, considerable right bundle-branch block, and right ventricular potentials of low amplitude. By radiologic examination marked cardiomegaly is seen, a contour consistent with right-sided enlargement, a narrow base, diminished pulmonary vascular markings, and poorly delineated pulmonary artery. Angiocardiography reveals a huge right atrial chamber with delayed emptying, poorly opacified pulmonary radicles, and frequent evidence of a right-to-left shunt. Cardiac catheterization demonstrates a large atrium, displacement of the tricuspid valve to the left, moderately elevated right atrial pressure, normal right ventricular systolic pressure and absence of a significant gradient across the pulmonary valve. Peripheral arterial O2 unsaturation is usual.