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一名淀粉样变性患者心脏和肝脏联合移植的麻醉管理:病例报告

Anesthetic management of a combined heart and liver transplantation in an amyloidotic patient: a case report.

作者信息

Barrio I M, Mtnez de Guereñu M A, Real M I, Del Campo I, Pérez-Cerdá F, Moreno E

机构信息

Department of Anesthesiology, Hospital Doce de Octubre, Madrid, Spain.

出版信息

Transplant Proc. 2007 Sep;39(7):2458-9. doi: 10.1016/j.transproceed.2007.07.058.

Abstract

Few cases of combined heart and liver transplantation (CHLT) have been reported for familial amyloidosis. Our first CHLT was performed on a female patient with familial amyloidosis due to a genetic defect in transthyretin, characterized by deposition of amyloid in various organs and tissues. This disease produced autonomic heart dysfunction that preceded the development of clinical manifestations and may be an important factor in determining the optimal timing for liver transplantation. CHLT can be performed successfully, even in patients with advanced disease. However, the most compromised patients are more exposed to intraoperative risks, postoperative complications, and worsening of extracardiac and extrahepatic symptoms. Our patient presented severe cardiac dysfunction requiring CHLT. The operative technique is far from being consolidated, despite this, both organs were transplanted in the same day with 2 hours in the intensive care unit (ICU) between surgeries. The outcome of both organs has been favorable. The amyloidotic liver was transplanted to another patient, a sequential (domino) transplantation.

摘要

家族性淀粉样变性的心脏和肝脏联合移植(CHLT)病例报道较少。我们的首例CHLT手术是在一名患有家族性淀粉样变性的女性患者身上进行的,该疾病由转甲状腺素蛋白的基因缺陷引起,其特征是淀粉样物质在各种器官和组织中沉积。这种疾病会导致自主神经心脏功能障碍,在临床表现出现之前就已存在,可能是决定肝移植最佳时机的重要因素。即使是晚期疾病患者,CHLT也能成功进行。然而,病情最严重的患者更容易面临术中风险、术后并发症以及心外和肝外症状的恶化。我们的患者出现严重心脏功能障碍,需要进行CHLT。尽管手术技术尚未成熟,但两个器官在同一天进行了移植,两次手术之间在重症监护病房(ICU)停留了2小时。两个器官的移植结果都很理想。淀粉样变性的肝脏被移植给了另一名患者,即进行了序贯(多米诺)移植。

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