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

• Published in: Pediatric transplantation 2011-05, Vol.15 (3), p.e47-e52
• Main Authors: Hori, Tomohide, Yonekawa, Yukihide, Okamoto, Shinya, Ogawa, Kohei, Ogura, Yasuhiro, Oike, Fumitaka, Takada, Yasutsugu, Egawa, Hiroto, Nguyen, Justin H., Uemoto, Shinji
• Format: Article
• Language: English
• Subjects: Abernethy malformation; Blood Flow Velocity; Child; Child, Preschool; congenital malformation; Fatty Liver - pathology; Hepatic Artery - pathology; hepato‐pulmonary syndrome; Humans; Hypertension, Pulmonary - surgery; Hypertension, Pulmonary - therapy; Liver - surgery; liver transplantation; Liver Transplantation - methods; Living Donors; Male; Portal Vein - abnormalities; Portal Vein - diagnostic imaging; Portal Vein - surgery; portosystemic shunt; pulmonary hypertension; Radiography; Time Factors; Treatment Outcome; Vascular Malformations - surgery
• ISSN: 1397-3142; 1399-3046
• DOI: 10.1111/j.1399-3046.2009.01269.x

Hori T, Yonekawa Y, Okamoto S, Ogawa K, Ogura Y, Oike F, Takada Y, Egawa H, Nguyen JH, Uemoto S. Pediatric orthotopic living‐donor liver transplantation cures pulmonary hypertension caused by Abernethy malformation type Ib.
Pediatr Transplantation 2011: 15:e47–e52. © 2010 John Wiley & Sons A/S. :  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.