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

Tomohide Hori, Yukihide Yonekawa, Shinya Okamoto, Kohei Ogawa, Yasuhiro Ogura, Fumitaka Oike, Yasutsugu Takada, Hiroto Egawa, Justin H. Nguyen, Shinji Uemoto

Research output: Contribution to journalArticle

13 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)e47-e52
JournalPediatric Transplantation
Volume15
Issue number3
DOIs
StatePublished - May 1 2011

Keywords

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

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Transplantation

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