Pediatric orthotopic living-donor liver transplantation cures pulmonary hypertension caused by Abernethy malformation type Ib

作者：Hori Tomohide^*; Yonekawa Yukihide; Okamoto Shinya; Ogawa Kohei; Ogura Yasuhiro; Oike Fumitaka; Takada Yasutsugu; Egawa Hiroto; Nguyen Justin H; Uemoto Shinji

来源：Pediatric Transplantation, 2011, 15(3): e47-e52.

DOI：10.1111/j.1399-3046.2009.01269.x

• congenital malformation
• Abernethy malformation
• portosystemic shunt
• hepato-pulmonary syndrome
• pulmonary hypertension
• liver transplantation

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.

• 出版日期2011-5