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复杂先天性心脏病患者的单纯肝移植:病例报告及文献综述

Liver-only Transplantation in a Patient With Complex Congenital Heart Disease: Case Report and Review of the Literature.

作者信息

Perquy David, Pirenne Jacques, Van De Bruaene Alexander, Rex Steffen, van Malenstein Hannah

机构信息

Department of Abdominal Transplant Surgery, University Hospitals Leuven, Leuven, Belgium.

Department of Cardiovascular Disease, University Hospitals Leuven, Leuven, Belgium.

出版信息

Transplant Direct. 2021 Jan 7;7(2):e649. doi: 10.1097/TXD.0000000000001096. eCollection 2021 Feb.

Abstract

BACKGROUND

Patients with liver failure due to or in addition to congenital heart disease (CHD) represent a growing population in need of organ transplantation. Traditionally, these patients received a combined heart and liver transplantation carrying a high risk of perioperative morbidity and mortality.

METHODS

We discuss a patient with complex cyanotic CHD and biliary atresia undergoing liver-only transplantation. Furthermore, a literature study was performed on combined congenital heart and liver disease in the setting of transplantation.

RESULTS

We describe a unique case of a patient with severe CHD undergoing orthotopic liver transplantation for biliary atresia. In the literature, congenital malformations affecting different organs seems not that infrequent. Liver-only transplantation has been described in mild CHD, although data in adult patients are scarce. In severe CHD, the liver usually suffers from congestion. The severity of liver disease and reversibility should be estimated to decide on combined heart-liver transplantation.

CONCLUSIONS

Our case and a review of the literature demonstrate that a patient-tailored approach with liver-only transplantation may be an appropriate alternative to combined heart and liver transplantation in selected cases.

摘要

背景

因先天性心脏病(CHD)导致或合并肝功能衰竭的患者群体日益庞大,他们需要器官移植。传统上,这些患者接受心脏和肝脏联合移植,围手术期发病率和死亡率风险很高。

方法

我们讨论了一名患有复杂青紫型CHD和胆道闭锁的患者接受单纯肝脏移植的情况。此外,还对移植背景下先天性心脏病和肝脏疾病的合并情况进行了文献研究。

结果

我们描述了一例患有严重CHD的患者因胆道闭锁接受原位肝脏移植的独特病例。在文献中,影响不同器官的先天性畸形似乎并不罕见。轻度CHD患者已报道过单纯肝脏移植,尽管成年患者的数据较少。在严重CHD中,肝脏通常会出现充血。应评估肝脏疾病的严重程度和可逆性,以决定是否进行心脏-肝脏联合移植。

结论

我们的病例及文献回顾表明,在某些特定情况下,针对患者量身定制的单纯肝脏移植方法可能是心脏和肝脏联合移植的合适替代方案。

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