P04.05: Atypical inferior vena cava and ductus venous blood flow velocity pattern in the fetus with Ebstein's anomaly: a case report

Abstract

We present a case of Ebstein’s anomaly with tri-phasic inferior vena cava (IVC) and ductus venosus (DV) forward flow pattern. Pulsed tissue Doppler imaging (TDI) were performed to elucidate the relationship of the atypical venous flow and the ventricular wall motions. A 32 year-old nulliparous woman conceived a monochorionic-diamniotic twin pregnancy by IVF. On a routine fetal sonography at 14 weeks of gestation, Twin A showed cardiomegaly (cardio-thoracic ratio: 53%) and severe tricuspid regurgitation (TR). Another twin was sonographically unremarkable. With advance of gestation, fetal echocardiography on Twin A revealed right atrial enlargement, severe holosystolic TR (1.3m/sec), the enlarged sail-like anterior leaflet of tricuspid valve (TV), and retrograde pulmonary artery and ductal flow in the absence of pulmonary regurgitation. Posterior and septal leaflets of TV were not detected. For sonographic findings stated above, Ebstein’s anomaly and pulmonary atresia was prenatally diagnosed. Pulsed Doppler examination showed tri-phasic IVC and DV forward flow pattern. Pulsed TDI recording of the right atrioventricular valve ring showed abnormal shortening after S wave from late systolic phase to isovolumic relaxation phase of the cardiac cycle. This abnormal shortening synchronized with the second wave of IVC and DV forward flow, and the left ventricle showed the same shortening. There were no sign of fetal arrhythmia or hydrops fetalis. A Cesarean section was performed at 36 weeks of gestations. Twin A was a female infant weighing 2290g with Apgar scores of 2 and 6 at 1 and 5 minutes, respectively. Postnatal diagnosis was the same as prenatal assessment. Starnes operation was performed at 6th day after birth. But she died at the 7th day because of cardiac failure. In conclusion, we detected tri-phasic IVC and DV forward flow pattern in the fetus with Ebstein’s anomaly. We speculate that the abnormal ventricular motion caused the atypical venous flow pattern.