A new staging system for cardiac transthyretin amyloidosis
A new staging system for cardiac transthyretin amyloidosis
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DOI：
10.1093/eurheartj/ehx589
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发表时间：
2018-08-07
影响因子：
39.3
通讯作者：
Hawkins, Philip N.
中科院分区：
文献类型：
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作者：
Gillmore, Julian D.;Damy, Thibaud;Hawkins, Philip N.
目的心脏转甲状腺素(ATTR)淀粉样变性是一种日益被认识的、进行性和致命性的心肌病，其自然病史尚不清楚。我们试图建立并验证一种新的预后分期系统，该系统同时适用于野生型和遗传性变异型ATTR型淀粉样心肌病患者。方法与结果869例在英国国家淀粉样变性中心就诊的心脏ATTR型淀粉样变性患者，根据两个普遍测量的生物标志物的切点，将其分为三个疾病阶段：N末端B型利钠肽(NT-ProBNP)和估计的肾小球滤过率(EGFR)。I期定义为NT-ProBNP=45ml/min，II期定义为NT-ProBNP和GT；3000 ng/L和表皮生长因子受体
Aims Cardiac transthyretin (ATTR) amyloidosis is an increasingly recognized, progressive, and fatal cardiomyopathy, the natural history of which remains unclear. We sought to establish and validate a new prognostic staging system applicable to patients with both wild-type ATTR (ATTRwt) and hereditary variant ATTR (ATTRv) amyloid cardiomyopathy.Methods and results Eight hundred and sixty-nine patients with cardiac ATTR amyloidosis (553 with ATTRwt and 316 with ATTRv) attending the UK National Amyloidosis Centre were stratified into three disease stages at baseline on the basis of cut points in two universally measured biomarkers, N-terminal pro-B-type natriuretic peptide (NT-proBNP) and estimated glomerular filtration rate (eGFR). Stage I was defined as NT-proBNP = 45 ml/min, Stage Ill was defined as NT-proBNP >3000 ng/L and eGFR