[Perioperative management of two neonates with severe Ebstein's anomaly with pulmonary atresia]

Ebstein's anomaly appearing during the neonatal period carries a high mortality rate. We report the perioperative management of two neonates with severe Ebstein's anomaly associated with pulmonary atresia. Their chest radiography revealed massive cardiomegaly, with cardiothoracic ratio of 90% and 100%, respectively. Their tracheas were intubated immediately after birth because of severe respiratory distress. The babies were laid in the prone position and one of them was managed with high frequency oscillatory ventilation (HFO) for prevention of pulmonary barotrauma and extensive atelectasis in the preoperative period. On the 4th and 3rd day after birth, respectively, they underwent Starnes procedure which consists of closure of tricuspid valve, enlargement of atrial septal defect, reduction of right atrium and creation of aortopulmonary shunt reported to be a useful palliative treatment for critically ill neonates with Ebstein's anomaly. Case 1 baby could not be weaned from cardiopulmonary bypass because of low cardiac output. Case 2 could not be weaned from extracorporeal membrane oxygenation due to hypoxia. We consider, however, treatment of respiratory failure and lung protection after birth are important for the prognosis. Perioperative use of HFO may be advantageous for the neonate with severe Ebstein's anomaly with pulmonary atresia.