Living related donor liver transplantation in a patient with severe aorticstenosis

We report the successful anaesthetic management of a young girl with Alagil le's syndrome and severe aortic stenosis (resting pressure gradient 88 mm H g) undergoing living related donor liver transplantation (LRDLT). The patie nt had end-stage liver disease and LRDLT was performed before replacement o f the aortic valve. Anaesthesia was conducted uneventfully with the aid of a pulmonary artery catheter. Intra-aortic balloon pumping was used in the p erioperative period for protection against myocardial ischaemia. Total clam ping of the inferior vena cava was avoided during surgery and volume admini stration was guided by the pulmonary artery pressure. A stable circulation was maintained in the reperfusion period. The patient was discharged from h ospital on day 54 after operation with normal liver function. Two years lat er her aortic valve was replaced successfully.