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心肌病心力衰竭:欧洲心脏病学会心力衰竭协会立场文件。

Heart failure in cardiomyopathies: a position paper from the Heart Failure Association of the European Society of Cardiology.

机构信息

University of Belgrade Faculty of Medicine, Belgrade, Serbia.

Serbian Academy of Sciences and Arts, Belgrade, Serbia.

出版信息

Eur J Heart Fail. 2019 May;21(5):553-576. doi: 10.1002/ejhf.1461. Epub 2019 Apr 16.

Abstract

Cardiomyopathies are a heterogeneous group of heart muscle diseases and an important cause of heart failure (HF). Current knowledge on incidence, pathophysiology and natural history of HF in cardiomyopathies is limited, and distinct features of their therapeutic responses have not been systematically addressed. Therefore, this position paper focuses on epidemiology, pathophysiology, natural history and latest developments in treatment of HF in patients with dilated (DCM), hypertrophic (HCM) and restrictive (RCM) cardiomyopathies. In DCM, HF with reduced ejection fraction (HFrEF) has high incidence and prevalence and represents the most frequent cause of death, despite improvements in treatment. In addition, advanced HF in DCM is one of the leading indications for heart transplantation. In HCM, HF with preserved ejection (HFpEF) affects most patients with obstructive, and ∼10% of patients with non-obstructive HCM. A timely treatment is important, since development of advanced HF, although rare in HCM, portends a poor prognosis. In RCM, HFpEF is common, while HFrEF occurs later and more frequently in amyloidosis or iron overload/haemochromatosis. Irrespective of RCM aetiology, HF is a harbinger of a poor outcome. Recent advances in our understanding of the mechanisms underlying the development of HF in cardiomyopathies have significant implications for therapeutic decision-making. In addition, new aetiology-specific treatment options (e.g. enzyme replacement therapy, transthyretin stabilizers, immunoadsorption, immunotherapy, etc.) have shown a potential to improve outcomes. Still, causative therapies of many cardiomyopathies are lacking, highlighting the need for the development of effective strategies to prevent and treat HF in cardiomyopathies.

摘要

心肌病是一组异质性的心肌疾病,也是心力衰竭(HF)的重要病因。目前对于心肌病患者 HF 的发病率、病理生理学和自然史的认识有限,其治疗反应的独特特征也尚未得到系统研究。因此,本立场文件重点关注扩张型心肌病(DCM)、肥厚型心肌病(HCM)和限制型心肌病(RCM)患者 HF 的流行病学、病理生理学、自然史和最新治疗进展。在 DCM 中,射血分数降低的 HF(HFrEF)的发病率和患病率均较高,尽管治疗有所改善,但仍是最常见的死亡原因。此外,晚期 DCM 心力衰竭是心脏移植的主要适应证之一。在 HCM 中,射血分数保留的 HF(HFpEF)影响大多数梗阻性 HCM 患者,约 10%的非梗阻性 HCM 患者也会发生 HFpEF。及时治疗很重要,因为尽管 HCM 中晚期 HF 少见,但发展为晚期 HF 预示着预后不良。在 RCM 中,HFpEF 很常见,而 HFrEF 则在淀粉样变性或铁过载/血色病中更晚且更常见。无论 RCM 的病因如何,HF 均是预后不良的先兆。目前对心肌病中 HF 发生发展机制的认识进展对治疗决策具有重要意义。此外,新的病因特异性治疗方法(如酶替代疗法、转甲状腺素蛋白稳定剂、免疫吸附、免疫疗法等)显示出改善预后的潜力。然而,许多心肌病仍缺乏病因治疗方法,这突显了需要开发有效的策略来预防和治疗心肌病中的 HF。

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