Anaesthesia in a child with uncorrected aortopulmonary window for non-cardiac surgery

Aortopulmonary window (APW) is a rare congenital cardiac anomaly characterized by communication between ascending aorta and main pulmonary artery. It represents between 0.2% and 0.3% of all congenital cardiac lesions. The anaesthetic management of a child with severe pulmonary hypertension associated with right to left shunt is clearly a demanding situation.A13 year old boy presented for open reduction and internal fixation of right femur. The child was diagnosed with congenital heart disease, APW with severe irreversible Pulmonary Artery Hypertension (PAH). Anaesthetic management comprised of a balanced approach with administration of general anaesthesia and regional analgesia with perioperative invasive haemodynamic monitoring.