title: Successful living related liver transplantation in a case with biliary atresia associated with corrected transposition of the great arteries.
abstract: Little is known about the safety of LRLTx in children with end stage liver disease associated with congenital cardiac anomalies. We report the successful LRLTx in a case with extrahepatic biliary atresia associated with cTGA, VSD, and PS. Preoperative cardiac function was evaluated by cardiac echogram and cardiac catheterization. The recipient's cardiac function was preserved (EF; 54%); however, because of the left to right shunt disease, oxygen saturation was 91%. At operation, carbon dioxide insufflation into the abdominal cavity was attempted to prevent sudden air embolism. Hemodynamic variables were stabilized during partial clamping of the inferior vena cava, and at reperfusion of the portal vein. However, a sudden decrease in blood oxygenation was observed during hepatico-jejunostomy, which was easily normalized by graft mobilization. Post-operatively, neither heart failure nor cerebral infarction because of air embolism was observed. In conclusion, together with preserved cardiac function and carbon dioxide insufflation, LRLTx was successful. Further studies are required to establish the algorithm for the strategy of treating both congenital cardiac anomalies and liver failure.
authors.affiliation: Division of Pediatric Surgery, Department of Surgery, Osaka University Graduate School of Medicine, 2-2 Yamadaoka, Suita, Osaka 565-0871, Japan. kimutaku@pedsurg.med.osaka-u.ac.jp
authors.forename: Takuya
authors.initials: T
authors.lastname: Kimura
authors.forename: Toshimichi
authors.initials: T
authors.lastname: Hasegawa
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Ihara
authors.forename: Sotaro
authors.initials: S
authors.lastname: Mushiake
authors.forename: Shigetoyo
authors.initials: S
authors.lastname: Kogaki
authors.forename: Keizo
authors.initials: K
authors.lastname: Dono
authors.forename: Masahiro
authors.initials: M
authors.lastname: Fukuzawa
mesh_terms: D001656:Biliary Atresia / Q000150:complications*; D006348:Cardiac Surgical Procedures; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D017093:Liver Failure / Q000209:etiology / Q000601:surgery*; D016031:Liver Transplantation / Q000379:methods*; D013997:Time Factors; D014188:Transposition of Great Vessels / Q000150:complications* / Q000601:surgery