Ebstein's anomaly is a rare congenital defect in which the tricuspid valve is abnormally displaced downward towards the right ventricle. This implies that in this condition, the basal part of the right ventricle becomes atrialized. This results in a large right atrium, thereby increasing its capacitance. Sometimes the right atrium may be huge in size. The tricuspid valve may often be incompetent and lead to variable degrees of tricuspid regurgitation. In addition, the right ventricular systolic function will often be diminished. The effects of the diminished right ventricular function can be expected to decrease the x' descent. The tricuspid regurgitation, on the other hand, depending on its severity, can be expected to raise the right atrial v wave and cause the y descent to be exaggerated. Despite these changes in the right atrial pressure pulse contours, the jugulars may not reflect these adequately because of the large right atrial capacitance, which may have a buffering effect. In fact, the descents in the jugulars and thejugular pulsations may be hard to see even in the relatively young patients with this defect. It is important to consider a large sized right atrium with high capacitance effect as part of a differential diagnosis for an inconspicuous jugular contour in the young adult.
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