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Ebstein Anomaly
Ebstein anomaly is an anomaly that results from malformation and malposition of the tricuspid valve. The valve is displaced into the right ventricle, so that a portion of the right ventricle is contiguous with the right atrium. The tricuspid valve is dysplastic and incompetent, leading to tricuspid regurgitation and enlargement of the right atrium. During atrial systole, blood flows from the right atrium towards the apex of the right ventricle. During ventricular systole, the blood regurgitates from the portion of the right ventricle distal to the tricuspid valve back across the dysplastic tricuspid valve into the right atrium. Hydrops may develop in utero due to fetal cardiac failure.
The prognosis for this cardiac anomaly is poor, with 80% of infants dying in the perinatal period. The prognosis is particularly poor if hydrops develops in utero or when there is pulmonary hypoplasia as a result of compression of the lungs by the enlarged heart. Long-term survivors of Ebstein anomaly often suffer from persistent cardiac arrhythmias.

Sonography

With Ebstein anomaly, the four-chamber view of the heart is abnormal. The heart is markedly enlarged, and the right atrium is especially dilated. The tricuspid valve can be seen displaced towards the apex of the right ventricle, located within the right ventricle rather than at the atrioventricular junction. Tricuspid regurgitation can be demonstrated with color or spectral Doppler.




Ebstein anomaly: Transverse image of the thorax with a four-chamber view of the heart demonstrates a markedly enlarged right atrium (RA) and mildly enlarged right and left ventricles (RV and LV). The tricuspid valve (arrow) is displaced into the right ventricle below the level of the normally positioned mitral valve (arrowheads) (S, spine).







Ebstein anomaly: Four-chamber image of the heart demonstrates an enlarged right atrium (RA) and displacement of the tricuspid valve (arrow) into the right ventricle, leaving a small right ventricular chamber (RV) (LA, left atrium; LV, left ventricle).
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