Circular shunting of blood: a complication of neonatal Ebstein anomaly

We report a severe neonatal presentation of Ebstein anomaly with homodynamic aggravation at birth attributed to patent ductus arteriosus and circular shunt. Prenatal diagnosis of Ebstein anomaly of the tricuspid valve with functional pulmonary atresia was made at 37 weeks gestation by fetal two- and three-dimensional echocardiography. A cesarean section was performed in view of poor fetal tolerance. The newborn was born with hydrops and multivisceral failure. A post-natal echocardiography demonstrated a left-to-right shunt across the patent ductus arteriosus and functional pulmonary atresia with severe pulmonary insufficiency with absent forward flow. This created a circular shunt, where blood flowed through the ductus to the pulmonary arteries, retrograde through the pulmonary artery and Ebstein valve, across the patent foramen ovale and out the aorta. Prostaglandin E1 infusion was stopped, resulting in clinical and echocardiographic improvement. The management of the phenomenon of a circular shunt across a patent ductus arteriosus with Ebstein malformation involves promoting early ductal closure by stopping prostaglandin therapy.