EUR HEART J:心脏淀粉样变性——脂肪垫细针穿刺术的诊断敏感性

2017-06-25 MedSci MedSci原创

心内膜心肌刚果红染色活检是怀疑性心脏淀粉样变(CA)的诊断金标准。但该过程存在风险,虽然风险较小,却与严重的并发症有关联,并且由于该项诊断需要专业技术会延误诊断。相比之下,心脏脂肪垫细针穿刺(FPFNA)是一种简单的、安全且完善的系统性淀粉样变性手术,但其对疑似CA患者的诊断敏感性尚不清楚。

背景:
心内膜心肌刚果红染色活检是怀疑性心脏淀粉样变(CA)的诊断金标准。但该过程存在风险,虽然风险较小,却与严重的并发症有关联,并且由于该项诊断需要专业技术会延误诊断。相比之下,心脏脂肪垫细针穿刺(FPFNA)是一种简单的、安全且完善的系统性淀粉样变性手术,但其对疑似CA患者的诊断敏感性尚不清楚。

方法:
本研究共招募了600名患者,他们被诊断为心脏淀粉样变(CA):216例AL型淀粉样变、113例遗传性甲状腺运载蛋白(ATTRm)淀粉样变和271例野生型甲状腺运载蛋白(ATTRwt)淀粉样变。并评估了心脏脂肪垫细针穿刺(FPFNA)诊断心脏淀粉样变的敏感性。

结果:
181/216例(84%)AL型心脏淀粉样变患者通过刚果红染色FPFNAs检测淀粉样蛋白,通过SAP组件闪烁扫描法评估得出:共有100、97和78%分别为大的、中度和全身小淀粉样蛋白(P<0.001);通过免疫组织化学方法成功地确定AL型心脏淀粉样变102/216(47%)例。在51/113(45%) ATTRm CA患者的FPFNAs中发现了淀粉样蛋白,只有42 / 271(15%)病例有ATTRwt CA。

结论:
FPFNA对心脏的淀粉样变性有合理的诊断敏感性,特别是在具有大的全身淀粉样蛋白的患者中。虽然在甲状腺运载蛋白心脏淀粉样变中,尤其是ATTRwt ,FPFNA的诊断敏感性明显较低,但它可能有时会避免对心内膜心肌活检的需要。

原始出处
Candida Cristina Quarta, Esther Gonzalez-Lopez, Janet A.Gilbertson, et al. Diagnostic sensitivity of abdominal fat aspiration in cardiac amyloidosis. EUR HEART J. 2017 June.

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    2017-06-27 rgjl
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    2017-06-27 zhaojie88
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  5. 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  6. 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  7. 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    2017-06-27 huangdf
  8. 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Nat rev Cardiol:心脏磁共振在心脏淀粉样变中的应用价值

甲状腺素运载蛋白介导的心脏淀粉样变(ATTR)是一种致命性的疾病,其发病机制为甲状腺素运载蛋白野生型或突变型以淀粉样形式沉淀于心脏。尽管针对这种疾病的特定治疗正在出现,但疾病诊断以及疾病进展监测仍存在很大挑战。【原文下载】 来自英国的Fontana教授称:“淀粉样变进展期时心电图、超声心动图、生物标记物等指标有所改变,但这些指标可能会受到左心室肥厚等混杂因素的干扰。”2013年,研究报道