Perioperative management for open balloon atrial septostomy immediately after caesarean section in a baby with hypoplastic left heart syndrome and intact atrial septum

Hypoplastic left heart syndrome (HLHS) with intact atrial septum (IAS) is an extreme type of single ventricle physiology among congenital heart diseases, in which a baby cannot supply oxygenated blood into systemic circulation without alternative pathway. We report the case of the neonate undergoing open balloon atrial septostomy (BAS) and bilateral pulmonary artery banding (PAB) soon after scheduled caesarean sections (C/S). A 35-year-old female was pregnant and fetal echocardiography at 32 weeks revealed one of the twins as HLHS/IAS. Severe hypoxia soon after birth was suspected. Thus, scheduled C/S followed by open BAS was planned. At 36 weeks of gestation, the mother was anesthetized with spinal bupivacaine and the female baby with HLHS/IAS was delivered. After diagnosed definitely by pediatric cardiologists, her trachea was intubated by anesthegiologists and umbilical catheters were placed by neonatologists. Then the baby was transferred to neighboring operating theater for BAS 68 minutes after the birth, while her Sp_O2 was maintained around 75-85% through serial procedures. Open BAS and PAB were performed under general anesthesia without any hemodynamic instability or severe hypoxia. Cooperation among anesthegiologists, neonatologists, pediatric cardiologists, and cardiac surgeons is mandatory in order to successfully complete such a rushed procedure.