Surgical treatment for neonatal Ebstein's anomaly with circular shunting

Abstract

We report a case of surgical treatment for neonatal Ebstein's anomaly with circular shunting. A prenatal diagnosis of Ebstein's anomaly was made at 30 weeks of gestation. In addition to severe tricuspid regurgitation, severe pulmonary regurgitation was also noted, resulting in a 'circular shunt'. At 38 weeks and 2 days of gestation, the baby was delivered by Caesarean section. A two-stage palliative operation was planned. As the first palliation, the pulmonary artery was ligated 1 h after birth to eliminate the circular shunt. Lipo-prostaglandin E(1) was administrated continuously to keep the ductus arteriosus patent. Four days after first operation, modified Starnes operation was performed as the second-stage palliation. The tricuspid valve was closed by an ePTFE patch with 3-mm fenestration. Pulmonary blood flow was supplied by a right modified Blalock-Taussig shunt with a 3.5-mm diameter ePTFE graft. The infant's postoperative haemodynamic status was stable. The patient is being followed up carefully and is scheduled to have a univentricular repair in the future.