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多米诺肝移植16年后心脏转甲状腺素蛋白淀粉样变的多模态成像

Multimodality Imaging of Cardiac Transthyretin Amyloidosis 16 Years After a Domino Liver Transplantation.

作者信息

Bechiri M Y, Eliahou L, Rouzet F, Fouret P-J, Antonini T, Samuel D, Adam R, Adams D, Slama M S, Algalarrondo V

机构信息

AP-HP, Service de Cardiologie, Hôpital Béclère, Clamart, France.

AP-HP, Service de Médecine Nucléaire, Hôpital Bichat Claude Bernard, Paris, France.

出版信息

Am J Transplant. 2016 Jul;16(7):2208-2212. doi: 10.1111/ajt.13755. Epub 2016 Mar 17.

Abstract

We report the case of a 62-year-old man hospitalized in May 2015 for symptomatic heart failure. His medical history included two liver transplantations. The first liver transplantation was performed in 1999 for a mixed alcoholic and hepatitis C-related cirrhosis and the patient received the liver of another patient with Val30Met transthyretin amyloidosis using the domino technique. In 2008, he complained of neuropathic pains and an iatrogenic-acquired transthyretin amyloidosis was diagnosed. On cardiac evaluation, amyloidosis was suspected. In March 2010, a second liver transplantation was performed with a deceased donor without complication. In May 2015, a first episode of symptomatic heart failure occurred and cardiac amyloidosis was investigated by a multimodality evaluation. Electrocardiogram, cardiac biomarkers, echocardiography, and cardiac MRI were in favor of the diagnosis of amyloidosis, whereas Tc-dicarboxypropane diphosphonate scintigraphy was not. Endomyocardial biopsy finally confirmed the positive diagnosis of iatrogenic-acquired cardiac amyloidosis. This case is, to the best of our knowledge, the first to report biopsy-proven cardiac amyloidosis induced by domino liver transplantation and progressing heart failure in spite of retransplantation. The diagnostic modalities are discussed. This case should alert physicians to the cardiac risk in domino liver transplanted patients.

摘要

我们报告了一例2015年5月因症状性心力衰竭住院的62岁男性病例。他的病史包括两次肝移植。第一次肝移植于1999年进行,用于治疗酒精性和丙型肝炎相关的混合性肝硬化,患者使用多米诺技术接受了另一名患有Val30Met转甲状腺素蛋白淀粉样变性病患者的肝脏。2008年,他主诉神经性疼痛,被诊断为医源性获得性转甲状腺素蛋白淀粉样变性病。心脏评估时怀疑有淀粉样变性病。2010年3月,进行了第二次肝移植,供体为脑死亡者,无并发症。2015年5月,出现了首次症状性心力衰竭发作,并通过多模态评估对心脏淀粉样变性病进行了检查。心电图、心脏生物标志物、超声心动图和心脏磁共振成像均支持淀粉样变性病的诊断,而锝-二羧丙烷二膦酸盐闪烁扫描则不支持。心内膜活检最终证实了医源性获得性心脏淀粉样变性病的阳性诊断。据我们所知,该病例是首例经活检证实由多米诺肝移植引起的心脏淀粉样变性病且尽管进行了再次移植仍进展为心力衰竭的病例。文中讨论了诊断方法。该病例应提醒医生注意多米诺肝移植患者的心脏风险。

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