TY - JOUR
T1 - Pediatric orthotopic living-donor liver transplantation cures pulmonary hypertension caused by Abernethy malformation type Ib
AU - Hori, Tomohide
AU - Yonekawa, Yukihide
AU - Okamoto, Shinya
AU - Ogawa, Kohei
AU - Ogura, Yasuhiro
AU - Oike, Fumitaka
AU - Takada, Yasutsugu
AU - Egawa, Hiroto
AU - Nguyen, Justin H.
AU - Uemoto, Shinji
PY - 2011/5/1
Y1 - 2011/5/1
N2 - 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.
AB - 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.
KW - Abernethy malformation
KW - congenital malformation
KW - hepato-pulmonary syndrome
KW - liver transplantation
KW - portosystemic shunt
KW - pulmonary hypertension
UR - http://www.scopus.com/inward/record.url?scp=79954452158&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=79954452158&partnerID=8YFLogxK
U2 - 10.1111/j.1399-3046.2009.01269.x
DO - 10.1111/j.1399-3046.2009.01269.x
M3 - Article
C2 - 20136726
AN - SCOPUS:79954452158
SN - 1397-3142
VL - 15
SP - e47-e52
JO - Pediatric Transplantation
JF - Pediatric Transplantation
IS - 3
ER -