EP09.14: Surgical treatment for neonatal Ebstein's anomaly with circular shunting

Discussion and Conclusion Circular shunting results in blood flow that does not contribute to the systemic circulation, causing low output syndrome and multiple organ failure. Surgical treatment must be performed to salvage this fatal stateSimpson Andrews Sharland (SAS) scores and the Celermajer index are relatively wellestablished scoring methods for predicting the prognoses of EA1,2.TRIPP score3 included TR Introduction Ebstein’s anomaly (EA) is a malformation of the tricuspid valve. The clinical symptoms vary in severity depending on the anatomical malformation; severe tricuspid regurgitation (TR) and right ventricular hypoplasia produce a critical state in the neonate. Concomitant pulmonary regurgitation (PR) is rare, with reversal of blood flow to the right ventricle, resulting in a ‘circular shunt’ (Fig1). Case Report A prenatal diagnosis of EA was made at 34 weeks and 5 days of gestation because of cardiomegaly (Fig2). In addition to severe TR, moderate PR was noted (Fig3), resulting in a ‘circular shunt’. Cardiothoracic area ratio, TR velocity and left ventricular(LV) Tei index were showen inTable1. Because pleural and pericardial effusion appeared, the baby was delivered by Caesarean section at 35 weeks and 6 days of gestation. Postnatal course The baby was born with almost wall to wall heart (Fig 4).On the day of birth, the pulmonary trunk ligation and bilateral pulmonary artery banding was performed to eliminate a circular shunt. Lipo-prostaglandin E1 was administrated continuously to keep the ductus arteriosus patent. Six days after the operation, Starnes operation was performed. After that, the infant became stable and was discharged at 69 days after birth. RA