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Tethering of Tricuspid Valve Resulting from Aberrant Tendinous Cords Mimic Ebstein's Anomaly, Three‐Dimensional Echocardiography Approach

A neonate with refractory cyanosis, respiratory distress, and severe cardiomegaly was referred to our institution for suspicion of Ebstein’s anomaly. The electrocardiogram (Nihon Kohden, Tokyo, Japan) showed right atrial enlargement with incomplete right bundle branch block without preexcitation. Chest radiography revealed extreme cardiomegaly with decrease pulmonary vascularity. Two-dimensional echocardiography (2DE) revealed right atrial enlargement, tethering of tricuspid valve (TV), dilated TV annulus with severe regurgitation, and right to left shunt through a large atrial septal defect. We used real time three-dimensional echocardiography (RT3DE) Philips iE33 system (Andover, MA, USA), to assess more precisely the mechanism of the TV regurgitation; the region of ineffective coaptation was well visualized using the en face view. The movements of the septal and inferior leaflets were restricted by aberrant tendinous cords, (Figs. 1 and 2). The postprocessing analysis of the 3D volume dataset by multiplanar review mode (QLab 9, Philips, Andover, MA, USA) allowed visualization of the normal delamination of the septal and inferior leaflets without apical displacement of their hinge points (Fig. 3). The aberrant tendinous cords were clearly identified by sliding the planes throughout the leaflets.