Correction of Severe Ebstein's Anomaly in a Neonate with Pulmonary Atresia / 日本心臓血管外科学会雑誌

ABSTRACT

We report the successful surgical treatment of severe Ebstein's anomaly in a female neonate with pulmonary atresia. Soon after birth, a heart murmur was audible and the baby became cyanotic. Echocardiography showed Ebstein's anomaly with pulmonary atresia. We started a LipoPGE 1 infusion for the open ductus arteriosus, but her uncontrollable heart failure necessitated surgery. Thus, when she was 8 days old, she was placed on cardiopulmonary bypass and we performed a triuspid valve orifice closure with right atrium plication and enlargement of the interatrial communication. She received a modified Blalock-Taussig shunt at the age of 60 days, but a prolonged mirulinone infusion was needed for her persistent heart failure. When she was 10 months old, we inserted a bidirectional Glenn shunt and she was discharged. Finally, when she was 30 months old, she had a total cavopulmonary connection. The patient is now asymptomatic, 18 months after her last operation.