ABSTRACT
Hypoplastic left heart syndrome (HLHS) is a lethal, congenital cardiac defect of neonates. It has been reported to occur in approximately 0.016 to 0.036 per 1000 live births and surgical palliation or cardiac transplantation is needed to treat this defect. This syndrome is more common in males and is associated with other congenital extra cardiac abnormalities, and these patients may need surgery for non-cardiac reasons. Here we report a neonate with HLHS and duodenal atresia who required emergency surgery due to intestinal obstruction with spinal anaesthesia. This case had experienced various challenges due to cardiac and extra-cardiac abnormalities. Surgery was performed under spinal anaesthesia.