Objective: Successful anaesthetic management of child with tricuspid atresia and functionally single ventricle, undergoing corrective surgery for ano rectal malformation.

Methods and results: 14 month old child, presented with ano-rectal malformation and cyanosis since birth. Echocardiography showed tricuspid atresia, large osteum secondum ASD with right to left shunt, dilated and hypertrophied LV with normal contractility, rudimentary RV, Pulmonary stenosis, and small inlet VSD. Child had undergone Modified BT shunt at the age of 4 months, which was patent and functioning well. SPO2 was 80-82% at rest and 65-70% during crying on room air. Haematocrit was 52% and other lab investigations were with in normal limits. Child was on oral ecosprin which was stopped 5 days preoperatively and heparin infusion was started and was stopped 6 hrs prior to surgery. The child was posted for definitive surgery of anorectal malformation, Anaesthetic technique included fentanyl, pancuronium and sevoflurane in air oxygen mixture. ECG, SPO2, Arterial blood pressure and central venous pressure were monitored. Arterial BP was maintained in the range of 80-85/40-45 mm Hg, Phenylephrine was kept ready to treat any episode of hypotension aggressively but was not required. CVP was maintained approx.12 mmHg. Surgery lasted for 3 hrs and child was extubated at the end of surgery. Hypoxemia, hypercarbia, acidosis and hypothermia were avoided.

Conclusion: Most important goal was to maintain shunt patency and flow as this was the only source of pulmonary blood flow and oxygenation. It was also important to maintain the delicate balance between PVR and SVR.