[Circular shunt in the severe neonatal form of Ebstein's Anomaly. The prostaglandine infusion is it beneficial or harmful?]

Ebstein's disease with functional pulmonary atresia is a severe neonatal presentation of Ebstein's anomaly where the therapeutic management is typically based on the prescription of prostaglandins. The circular shunt is a serious "hemodynamic" complication which is often undiagnosed leading to the discontinuation of prostaglandins. We report a severe neonatal form of Ebstein's anomaly with hemodynamic deterioration relatted to a circular shunt. The diagnosis of Ebstein's anomaly with functional pulmonary atresia was made prenatally at 36 weeks of pregnancy. The patient was born at 38 weeks of gestation by caesarean section. Postnatal ultrasound confirmed the diagnosis. Treatment with prostaglandins was originally created to maintain the vital ductus arteriosus patent. Despite this treatment, hemodynamic deterioration was observed. Ultrasound monitoring showed pictures for a circular shunt. Indeed, blood coming into the pulmonary artery by the wide ductus arteriosus, was "drawn" to the right ventricle and the right atrium due to tricuspid regurgitation and from there to the left heart via the fossa ovalis shunting right to left, when it was ejected into the aorta and the ductus arteriosus. Before this circular shunt, treatment with prostaglandin was discontinued and treatment to reduce pulmonary resistance was described. However, the patient died prior to initiation of treatment. The neonatal form of Ebstein's anomaly is a severe form that can be complicated by a circular shunt. This hemodynamic phenomenon encourages early closure of the ductus arteriosus against indicating the prescription of prostaglandins.