Advancement of medical therapy and surgical techniques has improved the survival of patients with complex congenital cardiac abnormalities, resulting in more adult patients with Fontan physiology presenting for non-cardiac surgery.

A 24 years old young female weighing 44 kg, was scheduled for revision of sternal keloid scar. As a neonate, she had BT shunt performed and further at the age of three, modified Fontan’s procedure was accomplished. Her current cardiac lesions included pulmonary atresia with intact ventricular septum (PAIVS) and a large ASD with hypoplastic right ventricle.

Our monitoring included invasive arterial line, regular standard monitoring and transesophageal echocardiography. After adequate preloading, anaesthesia was induced with midazolam, fentanyl and etomidate titrated to effect. Intubation was facilitated after curarization using rocuronium. Maintainence was with sevoflurane in oxygen-air mixture. We maintained tidal volume of 5 ml/kg and respiratory rate of 14-16 so as to achieve normocapnia. Once anaesthetised TEE probe was inserted and assessment was made for chambers, valves and congenital defects to guide further fluid management. At the end of 3-hour surgery, extubation was performed on table with ease.

An imbalance between systemic vascular resistance, pulmonary vascular resistance, preload, cardiac rhythm and LV function may result in poor haemodynamics in these patients presenting for non-cardiac operations.

We report a successful outcome of this complicated congenital heart disease for non-cardiac surgery. This highlight: 1.Importance of having knowledge on Fontan physiology for such patients presenting for non-cardiac surgery.2.Perioperative prophylaxis of infective endocarditis.3.Adequate optimisation of filling status and maintenance of systemic vascular resistance.4.Fine balance of anaesthesia, analgesia and curarization.