2023 Volume 52 Issue 4 Pages 216-220
Ebstein's anomaly is abnormality of the right ventricular and atrioventricular valves, and the severity varies depending on the morphology. Neonatal cases are in a critical state due to severe tricuspid regurgitation and right ventricle dysfunction. We report on two neonate cases in which we performed modified Starnes surgery using a rapid two-stage approach for the most severe clinical condition of Ebstein's anomaly with pulmonary valve regurgitation. The first patient was born at 39 weeks of gestation with a weight of 3,012 g by suction delivery. On day 7 of life, she underwent main pulmonary artery ligation, bilateral pulmonary artery banding and right atrial plication. However, because lactic acidosis had progressed after surgery, she underwent an emergency modified Starnes operation on day 8 of life. The second patient was born at 37 weeks of gestation with a weight of 2,528 g by emergency caesarean section because of fetal edema. Although she underwent main pulmonary artery ligation on the day of birth, acidosis progressed, and blood circulation did not improve. For this reason, she underwent a modified Starnes operation on her first day of life.
