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Living Related Donor Liver Transplantation In A Patient With Alagille's Syndrome With Severe Pulmonary Stenosis

Pelin KARAASLAN, Selma OZGUR, Tumay Uluda YANARAL, Esra KUTLU, Hamdi KARAKAYALI, Emine Arzu KOSE

Alagille's syndrome (AGS) is a multisystem disorder affecting liver, heart, face and skeletal system. Approximately 25% of AGS patients may need liver transplantation (LT) in childhood. Unfortunately AGS patients have multisystem pathologies and they should be carefully evaluated before LT. Especially congenital heart defects in AGS patients may cause hemodynamic effects perioperatively in transplantation surgery. In this case report we aimed to discuss successful anesthetic management for living related donor liver transplantation (LRDLT) in a patient with Alagille's syndrome and severe pulmonary stenosis. Successful anesthetic management of a growth retarded 11-months old, 5110 grams infant for LT is a challenge especially with coexisting cardiac pathologies. Complete preoperative evaluation and careful perioperative monitoring of the patient resulted in stable circulation.