title: Successful treatment of pulmonary hypertension secondary to congenital extrahepatic portocaval shunts (Abernethy type 2) by living donor liver transplantation after surgical shunt ligation.
abstract: In this report, we describe a living donor liver transplantation (LDLT) in a patient (7-year-old boy) with Abernethy type 2 congenital extrahepatic portocaval shunts (CEPS). This patient underwent a surgical shunt ligation as the first treatment for pulmonary hypertension; pulmonary hypertension was improved and controlled successfully 4 years after the first operation. However, pulmonary hypertension recurred gradually because of multiple intrahepatic portosystemic shunts; therefore, LDLT was performed as a radical treatment of intrahepatic portosystemic shunts. His pulmonary arterial pressure was also controlled 22 months after LDLT, the postoperative continuous intravenous prostaglandin I(2) (PGI(2)) treatment could be withdrawn successfully. We suggest that clinicians carefully follow up the recurrent portosystemic shunt and cardiopulmonary disorders secondary to Abernethy type 2 CEPS.
authors.affiliation: Department of Hepatobiliary Pancreas and Transplant Surgery, Kyoto University Graduate School of Medicine, Kyoto, Japan. itaku@kuhp.kyoto-u.ac.jp
authors.forename: Taku
authors.initials: T
authors.lastname: Iida
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Ogura
authors.forename: Hiraku
authors.initials: H
authors.lastname: Doi
authors.forename: Shintaro
authors.initials: S
authors.lastname: Yagi
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Kanazawa
authors.forename: Hisashi
authors.initials: H
authors.lastname: Imai
authors.forename: Seisuke
authors.initials: S
authors.lastname: Sakamoto
authors.forename: Shinya
authors.initials: S
authors.lastname: Okamoto
authors.forename: Shinji
authors.initials: S
authors.lastname: Uemoto
mesh_terms: D002648:Child; D002675:Child, Preschool; D011464:Epoprostenol / Q000008:administration & dosage; D005402:Fistula / Q000601:surgery; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000601:surgery*; D008026:Ligation; D016031:Liver Transplantation / Q000379:methods; D019520:Living Donors; D008297:Male; D011169:Portal Vein / Q000002:abnormalities; D011170:Portasystemic Shunt, Surgical / Q000379:methods; D014682:Vena Cava, Inferior / Q000002:abnormalities
chemical_list: D011464:Epoprostenol