PMC8601841_01
A 4-chamber view in a fetal echocardiogram showing a heavily trabeculated spongy looking LV suggestive of non-compaction (arrow). It also shows the Ebstein's anomaly and a VSD (*) between the LV to the atrialized portion of the RV.
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The fetal echocardiogram (ECHO) was significant for EA, hypoplastic RV, a septal defect between the left ventricle and the atrialized portion of the RV (Gerbode-like defect) in addition to possible LVNC (Figure 1).
Transthoracic echocardiogram with 2D and color Doppler showing an apical displacement of septal leaflet of the tricuspid valve (arrow) and atrialization of RV. A left to right shunt is also seen across a VSD into the atrialized RV.
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These findings were confirmed by postnatal echocardiography which also showed a moderate to severe degree of RV outflow tract obstruction, multiple small ventricular septal defects, and left ventricular noncompaction (Figure 2).
Short axis cine SSFP image of hypoplastic RV, VSD (*) and normal sized LV. Note the abnormal trabeculations along the lateral and apical LV walls.
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As seen in Figure 3, the RV was severely hypoplastic, with an RV indexed end diastolic volume of 32 ml/m2 and normal systolic function with an ejection fraction of 58%.
An oblique coronal image from contrast enhanced cardiac CT showing a right modified Blalock-Taussig shunt (*), dilated right pulmonary artery (RPA), left atrium, and LV. Note the abnormal LV trabeculations along the LV inferior, lateral and apical walls.
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The severely dilated right atrium with an atrialized right ventricle was also redemonstrated (Figures 4 and 5).
A coronal cine SSFP image from cardiac magnetic resonance of the trabecular, hypoplastic RV and the large atrialized portion of the RV. The dilated right atrial appendage (RAA) is also seen.
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The severely dilated right atrium with an atrialized right ventricle was also redemonstrated (Figures 4 and 5).