Amyloidosis in Heart Failure
Amyloidosis in Heart Failure
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DOI：
10.1007/s11897-019-00446-x
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发表时间：
2019-12-01
影响因子：
--
通讯作者：
Stoerk, Stefan
中科院分区：
文献类型：
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作者：
Ihne, Sandra;Morbach, Caroline;Stoerk, Stefan
目的淀粉样变性是一种日益被人们认识但仍经常被忽视的心力衰竭原因。鉴于许多治疗轻链(AL)淀粉样变的有效方法和对转甲状腺蛋白(ATTR)淀粉样变有希望的新的治疗选择，需要提高照顾者的意识，以筛选淀粉样变作为一种重要的和潜在的可治疗的鉴别诊断。这篇综述概述了心脏淀粉样变性的多样性，它与心力衰竭的关系，诊断算法，以及根据淀粉样变性的潜在类型而应应用的治疗考虑。最近的研究发现，在缺乏单抗成分的情况下，非活检诊断在ATTR淀粉样变性中是可行的，从而导致更高的心脏ATTR淀粉样变性的检出率。生物标记物指导的分期系统已经更新，以便根据目前可用的独立于地区差异的生物标记物进行风险分层，但尚未进行前瞻性测试。遗传性和野生型ATTR淀粉样变性的新疗法越来越多。AL淀粉样变性的复杂治疗方案正在不断改进，导致更好的生存和生活质量。晚期心脏淀粉样变性的死亡率仍然很高，强调了早期诊断和治疗的重要性。心脏淀粉样变性的特点是病因和临床的异质性，导致经常延误诊断和不适当的高死亡风险。对于这种迄今部分无法治疗的疾病，新的治疗方案已经并将成为可用的，但对其实施提出了挑战。转诊到专门的中心，提供获得广泛和有针对性的诊断调查和治疗启动的机会，可能有助于应对这些挑战。
Purpose Amyloidosis represents an increasingly recognized but still frequently missed cause of heart failure. In the light of many effective therapies for light chain (AL) amyloidosis and promising new treatment options for transthyretin (ATTR) amyloidosis, awareness among caregivers needs to be raised to screen for amyloidosis as an important and potentially treatable differential diagnosis. This review outlines the diversity of cardiac amyloidosis, its relation to heart failure, the diagnostic algorithm, and therapeutic considerations that should be applied depending on the underlying type of amyloidosis. Recent Findings Non-biopsy diagnosis is feasible in ATTR amyloidosis in the absence of a monoclonal component resulting in higher detection rates of cardiac ATTR amyloidosis. Biomarker-guided staging systems have been updated to facilitate risk stratification according to currently available biomarkers independent of regional differences, but have not yet prospectively been tested. Novel therapies for hereditary and wild-type ATTR amyloidosis are increasingly available. The complex treatment options for AL amyloidosis are improving continuously, resulting in better survival and quality of life. Mortality in advanced cardiac amyloidosis remains high, underlining the importance of early diagnosis and treatment initiation. Cardiac amyloidosis is characterized by etiologic and clinical heterogeneity resulting in a frequently delayed diagnosis and an inappropriately high mortality risk. New treatment options for this hitherto partially untreatable condition have become and will become available, but raise challenges regarding their implementation. Referral to specialized centers providing access to extensive and targeted diagnostic investigations and treatment initiation may help to face these challenges.