Recognition and Initial Management of Fulminant Myocarditis A Scientific Statement From the American Heart Association
Recognition and Initial Management of Fulminant Myocarditis A Scientific Statement From the American Heart Association
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DOI：
10.1161/cir.0000000000000745
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发表时间：
2020-02-11
期刊：
影响因子：
37.8
通讯作者：
Vardeny, Orly
中科院分区：
文献类型：
--
作者：
Kociol, Robb D.;Cooper, Leslie T.;Vardeny, Orly
暴发性心肌炎是一种罕见的综合征，其特征是突发性和严重的弥漫性心脏炎症，常因心源性休克、室性心律失常或多器官系统衰竭而导致死亡。从历史上看，FM几乎完全是在尸检中诊断出来的。根据定义，所有FM患者都需要某种形式的正性肌力或机械循环支持，以维持终末器官灌注，直到移植或恢复。FM的特定亚型可能对免疫调节治疗以及指南指导的医疗护理有反应。尽管循环支持、原位心脏移植和疾病特异性治疗的可用性不断增加，但由于诊断和循环支持启动延迟以及缺乏经过适当培训的专家来管理病情，FM患者的发病率和死亡率显著增加。本科学声明概述了管理FM频谱所需的资源，包括体外生命支持、经皮和耐用的心室辅助装置、移植能力以及晚期心力衰竭、心胸外科、心脏病理学、免疫学和传染病方面的专家。对最有可能首先遇到FM的一线提供者进行教育，对于及时获得适当资源的设施，预防多器官系统衰竭以及在疾病过程中尽早定制疾病特异性治疗至关重要。
Fulminant myocarditis (FM) is an uncommon syndrome characterized by sudden and severe diffuse cardiac inflammation often leading to death resulting from cardiogenic shock, ventricular arrhythmias, or multiorgan system failure. Historically, FM was almost exclusively diagnosed at autopsy. By definition, all patients with FM will need some form of inotropic or mechanical circulatory support to maintain end-organ perfusion until transplantation or recovery. Specific subtypes of FM may respond to immunomodulatory therapy in addition to guideline-directed medical care. Despite the increasing availability of circulatory support, orthotopic heart transplantation, and disease-specific treatments, patients with FM experience significant morbidity and mortality as a result of a delay in diagnosis and initiation of circulatory support and lack of appropriately trained specialists to manage the condition. This scientific statement outlines the resources necessary to manage the spectrum of FM, including extracorporeal life support, percutaneous and durable ventricular assist devices, transplantation capabilities, and specialists in advanced heart failure, cardiothoracic surgery, cardiac pathology, immunology, and infectious disease. Education of frontline providers who are most likely to encounter FM first is essential to increase timely access to appropriately resourced facilities, to prevent multiorgan system failure, and to tailor disease-specific therapy as early as possible in the disease process.