Successful management of living donor liver transplantation for biliary atresia with single ventricle physiology—from peri‐transplant through total cavopulmonary connection: A case report

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 under...

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Bibliographic Details
Published in:Pediatric transplantation 2018-05, Vol.22 (3), p.e13118-n/a
Main Authors: 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
Format: Article
Language:English
Subjects:
Aorta
Biliary atresia
Case reports
Catheters
Children
congenital heart disease
Coronary artery disease
Fontan
Heart diseases
Heart surgery
Heart transplantation
heterotaxy
Liver transplantation
Liver transplants
Physiology
Regurgitation
single ventricle physiology
Transplants & implants
Ventricle
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Summary: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 IIb, intermediate atrioventricular septal defect, tricuspid regurgitation grade III, coarctation of aorta, interrupted inferior vena cava, bilateral superior vena cava, and polysplenia syndrome. Following LDLTx, 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 LDLTx (2 years post–EC‐TCPC). We describe the details of the management of LTx in the presence of cardiac anomalies and report the long‐term cardiac and liver function, from peri‐LDLTx through EC‐TCPC completion.
ISSN:1397-3142
1399-3046
DOI:10.1111/petr.13118