Pediatric orthotopic living-donor liver transplantation cures pulmonary hypertension caused by Abernethy malformation type Ib.
A 3.3-yr-old boy was diagnosed with PH caused by a PSS of Abernethy malformation type Ib. After control of PH, he underwent OLDLT at 4.9 yr. His PV flowed directly into the confluence of the CCLMHV and the IVC. To shorten the anhepatic phase, hepatic arterial flow was partially maintained. Removal of the native liver began simultaneously with the graft harvest. The proximal PV was cut at the optimal point for reconstruction. The distal PV was cut at the concrescence of the PV and the CCLMHV. After IVC-plasty, the LHV of the graft was attached with an anterior patch by venous grafting and was then anastomosed to the IVC. Although the mPAP temporarily increased above the mean arterial pressure, mPAP was stable during OLDLT. FNH and steatosis were confirmed histopathologically. In summary, pediatric OLDLT was performed successfully in PH caused by PSS.
Department of Hepato-pancreato-biliary, Transplant and Pediatric Surgery, Kyoto University Hospital, Kyoto, Japan. horit@kuhp.kyoto-u.ac.jp
Tomohide
T
Hori
Yukihide
Y
Yonekawa
Shinya
S
Okamoto
Kohei
K
Ogawa
Yasuhiro
Y
Ogura
Fumitaka
F
Oike
Yasutsugu
Y
Takada
Hiroto
H
Egawa
Justin H
JH
Nguyen
Shinji
S
Uemoto
D001783:Blood Flow Velocity; D002648:Child; D002675:Child, Preschool; D005234:Fatty Liver / Q000473:pathology; D006499:Hepatic Artery / Q000473:pathology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000601:surgery* / Q000628:therapy; D008099:Liver / Q000601:surgery; D016031:Liver Transplantation / Q000379:methods*; D019520:Living Donors; D008297:Male; D011169:Portal Vein / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery; D011859:Radiography; D013997:Time Factors; D016896:Treatment Outcome; D054079:Vascular Malformations / Q000601:surgery