Anesthetic Experience of Pediatric Living Donor Liver Transplantation with Hepatopulmonary syndrome after Repaired Tetralogy of Fallot kazumasa Hiroi, Takashi matsusaki, Mizuho Kaneda, Tomoyuki Kazumasa, Ryuji Kaku, Hiroshi Morimatsu Department of Anesthesiology and Resuscitology, Okayama University Hospital 腎移植 3時間9分 Roc. total dose ;202mg Introduction & Challenging Point History of Present Illness Labo Data&Chest X-P &Perfusion Scintigraphy Liver transplantation after congenital heart disease was so challenging in terms of hemodynamic management. The diagnosis of Hepatopulmonary syndrome (HPS) and the adaptation of liver transplantation had been discussed carefully by cardiologist, transplant surgical team and anesthesiologists. Challenging Point Massive bleeding by comprehensive operative technique due to visceral heterotaxia Severe hypoxia due to heart failure with massive transfusion. Circulatory and respiratory dysfunction by reperfusion injury His hypoxia got worse and he newly needed home oxygen therapy (SpO2 82% with oxygen supplementation). He had no residual disease after a corrective surgery for tetralogy of Fallot by echocardiogram. Hepatopulmonary syndrome (HPS) was confirmed by a contrast-enhanced echocardiogram and a lung perfusion scan demonstrating the presence of significant intrapulmonary shunting. His progressing hypoxia due to HPS needed additional therapy and we thought that liver transplantation (LDLT from her mother) was the only way to rescue his life. Plt 115,000 /μl AST 83 U/l BNP 330.7 pg/ml PT-INR 1.14 ALT 51 Na 134 mmol/l APTT 36.2 sec ALP 1,405 K 4.0 TP 5.7 g/dl T-Bil 2.54 mg/dl Cl 106 Alb 3.5 D-Bil 1.35 Pulmonary Arterial Contrast Discusiion We have prepared for inhalational NO preventing form progressing hypoxia by massive transfusion or reperfusion injury. Our cardiovascular surgical staff waited in case of using cardiopulmonary bypass according to severe hypoxia or circulatory collapse. Anesthesia Record O2 (l/min) 6 1.1 Air (l/min) 1.9 Sevoflurane (%) 1 2 Fentanyl (mcg) 40 20 50 Case Description BP(mmHg) HR(/min) SpO2 (%) / total 600ml 2-years-10-month-old male Height 83cm Body weight: 9.2kg Diagnosis: Biliatry Atresia Medical History: Kasai surgery (day 46) corrective surgery for tetralogy of Fallot Other complication visceral heterotaxia, polysplenia, deficit of inferior vena cava bilateral superior vena cava Conclusion We have successfully managed living donor liver transplantation with congenital heart disease. It was important that perioperative team prepared for the predictable event of emergency in the perioperative care disease. × ◎ ◎ × 1 2 3 4 5 6 7 8 9 Bleeding(ml)l 240 430 730 1085 1260 1555 1850 RCC (ml) 50 FFP (ml) 40