P31.07: Maternal complex congenital cardiac defect and fetal tachyarrhythmia

Abstract

A 33-year-old woman, gravida 2 para 2, was refferd to our institution at 35 weeks’ gestation, because of polyhydramnios. Ultarasound examination revealed mild polyhydramnios (amniotic fluid index:25) and juxtaposition of the descending aorta and inferior vena cava. Fetal echocardiography demonstrated a common atrioventricular valve, double outlet right ventricle, pulmonary stenosis. Four pulmonary veins drained to a confluence behind the left atrium arising a vertical vein passed through the diaphragma and then connected to portal vein. On pulsed Doppler examination, the pulmonary veins and vertical vein showed continuous, mildly pulsatile flow. All these features were suggestive of right atrial isomserism with total anomalous pulmonary venous connection to the portal vein. Absence of the ductus venosus without liver bypass was suspected by meticulous color flow mapping of the portal circulation in various planes. A female infant was delivered weighing 2810g with Apgar scores of 8 and 8 at 1 and 5 minutes, respectively. Postnatal echocardiography confirmed the prenatal findings. On pulsed Doppler examination, the vertical vein showed non-pulsatile continuous flow. The infant underwent open heart surgery and the pulmonary confluence was connected to the left atrium. But she died at the second day because of cardiac failure. We speculate absence of the ductus venosus without liver bypass might worsend the prognosis of the infant with total anomalous pulmonary venous connection to the portal vein.