Successful management of living donor liver transplantation for biliary atresia with single ventricle physiology-from peri-transplant through total cavopulmonary connection: A case report.
Successful management of living donor liver transplantation for biliary atresia with single ventricle physiology-from peri-transplant through total cavopulmonary connection: A case report.
复制标题
活体肝移植治疗单心室生理学胆道闭锁的成功管理——从移植前后到全腔静脉肺连接：病例报告。
DOI：
10.1111/petr.13118
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发表时间：
2018
期刊：
影响因子：
--
通讯作者：
Kuroda T.
中科院分区：
文献类型：
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作者：
Yamada Y;Hoshino K;Oyanagi T;Gatayama R;Maeda J;Katori N;Fuchimoto Y;Hibi T;Shinoda M;Matsubara K;Obara H;Aeba R;Kitagawa Y;Yamagishi H;Kuroda T.
单心室生理学患儿的肺循环和体循环完全混合，需要分期手术以实现这些循环或Fontan循环的分离。单心室生理学显著增加了接受非心脏手术的儿童的死亡风险。由于单心室患者的肝移植特别具有挑战性，因此只有少数报告已发表。我们在此报告了一例8个月大的儿童患者的成功LDLTx，该患者患有胆道闭锁、异位和单心室生理学复杂心脏病。心脏畸形包括完全性肺静脉回流异常IIb型、中间型房室间隔缺损、三尖瓣反流III级、主动脉缩窄、下腔静脉中断、双侧上级腔静脉和多脾综合征。LDLTx后，患者在3岁时接受全腔静脉分流+ Damus‐Kaye‐Stansel，在5岁时完成心外全腔静脉连接（EC‐TCPC）; LDLTx后已过去7年（EC ‐TCPC后2年）。我们描述了在存在心脏异常的情况下LTx的管理细节，并报告了从EST-LDLTx到EC-TCPC完成的长期心脏和肝脏功能。
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.