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Successful management of living donor liver transplantation for biliary atresia with single ventricle physiology—from peri‐transplant through total cavopulmonary connection: A case report
Author(s) -
Yamada Yohei,
Hoshino Ken,
Oyanagi Takayuki,
Gatayama Ryohei,
Maeda Jun,
Katori Nobuyuki,
Fuchimoto Yasushi,
Hibi Taizo,
Shinoda Masahiro,
Matsubara Kentaro,
Obara Hideaki,
Aeba Ryo,
Kitagawa Yuko,
Yamagishi Hiroyuki,
Kuroda Tatsuo
Publication year - 2018
Publication title -
pediatric transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.457
H-Index - 69
eISSN - 1399-3046
pISSN - 1397-3142
DOI - 10.1111/petr.13118
Subject(s) - medicine , biliary atresia , liver transplantation , peri , transplantation , ventricle , living donor liver transplantation , connection (principal bundle) , cardiology , structural engineering , engineering
Children with single ventricle physiology have complete mixing of the pulmonary and systemic circulations, requiring staged procedures to achieve a separation of these circulations, or Fontan circulation. The single ventricle physiology significantly increases the risk of mortality in children undergoing non‐cardiac surgery. As liver transplantation for patients with single ventricle physiology is particularly challenging, only a few reports have been published. We herein report a case of successful LDLTx for an 8‐month‐old pediatric patient with biliary atresia, heterotaxy, and complex heart disease of single ventricle physiology. The cardiac anomalies included total anomalous pulmonary venous return type II b, intermediate atrioventricular septal defect, tricuspid regurgitation grade III , coarctation of aorta, interrupted inferior vena cava, bilateral superior vena cava, and polysplenia syndrome. Following LDLT x, the patient sequentially underwent total cavopulmonary shunt + Damus‐Kaye‐Stansel at 3 years of age and extracardiac total cavopulmonary connection ( EC ‐ TCPC ) completion at 5 years of age; 7 years have now passed since LDLT x (2 years post– EC ‐ TCPC ). We describe the details of the management of LT x in the presence of cardiac anomalies and report the long‐term cardiac and liver function, from peri‐ LDLT x through EC ‐ TCPC completion.