Sack Falk-Udo, Kristen Arnt, Goldschmidt Hartmut, Schnabel Philipp A, Dengler Thomas, Koch Achim, Karck Matthias
Department of Cardiac Surgery, University of Heidelberg, Heidelberg, Germany.
Eur J Cardiothorac Surg. 2008 Feb;33(2):257-62. doi: 10.1016/j.ejcts.2007.10.025. Epub 2007 Dec 21.
Cardiac amyloidosis (CA) is associated with a poor prognosis and a survival rate of less than 30% 2 years after clinical manifestation. Considered as a semi-malignant disease, CA is often a contraindication for HTx; however, depending on the type of CA, there are excellent treatment regimes that can be combined with HTx. In AL-amyloidosis, chemotherapy and stem cell transplantation are necessary and in TTR-amyloidosis, where the liver is the source of the pathologic protein, liver transplantation is recommended after HTx.
More than 60 patients with AL-amyloidosis and more than 25 patients with ATTR-amyloidosis have been investigated at our centre. Eighteen patients showed signs of end-stage heart failure. Four patients died within 1 month after listing for HTx. Seven patients with AL (mean age 41.8 years) and five patients with ATTR-amyloidosis (mean age 42.6 years) were successfully transplanted with an actual survival rate of 91.6%. One patient died 8 months after HTx due to infection. Five AL patients received chemotherapy and SCT and one ATTR patient was liver transplanted. Three AL patients showed complete remission of amyloidosis.
Cardiac amyloidosis is a potentially curative disease after HTx when combined with either chemotherapy and SCT or LiverTx depending on the type of the amyloidosis. Due to the natural course of the disease, urgent HTx after cardiac manifestation is mandatory. With this approach, excellent survival rates and even remission of the underlying disease is possible.
心脏淀粉样变性（CA）预后较差，临床表现出现后2年生存率低于30%。CA被视为一种半恶性疾病，通常是心脏移植（HTx）的禁忌证；然而，根据CA的类型，有一些出色的治疗方案可与HTx联合使用。在AL型淀粉样变性中，化疗和干细胞移植是必要的，而在TTR型淀粉样变性中，肝脏是病理性蛋白的来源，建议在HTx后进行肝移植。
我们中心对60多名AL型淀粉样变性患者和25多名ATTR型淀粉样变性患者进行了研究。18名患者出现终末期心力衰竭迹象。4名患者在列入HTx名单后1个月内死亡。7名AL型患者（平均年龄41.8岁）和5名ATTR型淀粉样变性患者（平均年龄42.6岁）成功接受了移植，实际生存率为91.6%。1名患者在HTx后8个月因感染死亡。5名AL型患者接受了化疗和干细胞移植，1名ATTR型患者接受了肝移植。3名AL型患者淀粉样变性完全缓解。
根据淀粉样变性的类型，心脏淀粉样变性在HTx后结合化疗和干细胞移植或肝移植是一种潜在可治愈的疾病。由于该疾病的自然病程，心脏出现症状后紧急进行HTx是必要的。采用这种方法，有可能获得出色的生存率，甚至使潜在疾病缓解。
