Alida L. P. Caforio教授 意大利帕多瓦大学

　　<International Circulation>: The position paper on myocardial and pericardial diseases was published this July. Can you talk about the impact of this position paper on clinical practice?

　　Prof Caforio: Hopefully it will have a good impact because it was our intention to design it for the clinician to gain orientation in terms of the clinical suspicion of myocarditis as well as the diagnostic workup of myocarditis. There are two main messages in this paper which is quite comprehensive even for clinical cardiologists and not just molecular-oriented. The first is a review of the current knowledge of the disease from etiology to therapy. Secondly， there are new diagnostic criteria for suspected clinical myocarditis. The problem with myocarditis is that it has a very heterogeneous presentation and it can mimic many other diseases. In general， there are some more common presentations: a pseudo-infarction presentation with or without troponin increase and normal coronary arteries; a heart failure presentation that can be acute or chronic with or without a dilated heart; another possibility is an arrhythmia presentation either mild with palpitations or more severe with syncope and even unexpected sudden cardiac death or aborted sudden cardiac death; and then the most feared is a life-threatening， acute， so-called fulminant presentation which may involve cardiogenic shock. These are the main clinical presentations. However， myocarditis is a diagnosis of exclusion. You first have to exclude coronary artery disease and second， known causes for this syndrome. So diagnosis cannot only be based on the clinical presentation. The suspicion is clinical. You need an ECG. On ECG you may have many different alterations which are detailed in the tables in the paper. You can have ECG or Holter or stress test findings and you may have altered functional and structural findings on cardiac imaging， either non-invasive or invasive (echo， CMR or angiography). These may be segmental or global. You may even have a normal ejection fraction and still have myocarditis. Of course you may have CMR tissue characterization that is suggestive of myocarditis. The new criteria say that you need a plausible clinical presentation plus one or more of the ECG or other instrumental findings from different categories in order to have a clinical suspicion of myocarditis. If there are no symptoms and diagnosis is occasional then you need more instrumental findings to clinically suspect myocarditis， but it is still only a suspicion. To have a confirmed diagnosis， you need to exclude coronary artery disease and second， have an endomyocardial biopsy. On the basis of endomyocardial biopsy， where not only histological criteria are required， but also special staining which involves immunohistochemistry and a search by molecular methods for infectious agents， you can distinguish different types of myocarditis. All of the autoimmune-mediated forms are infection negative. Then you may have infectious forms and there is a very small proportion where you may have both an infectious agent and immune activation. Fortunately this is a tiny group; the majority fall into the infectious or autoimmune subsets. For the next step， there are a lot of recommendations in relation to the validity and usefulness of the various tests including invasive and non-invasive tests. The aim is to reach a etiopathogenic diagnosis of myocarditis because special autoimmune forms can be managed and treated with safe immune suppression. Vice versa， if an infectious agent is there， depending on the infectious agent， the immunosuppression is contraindicated and there may be different options. This is a fairly comprehensive document and most importantly is a consensus document of the whole European myocarditis and cardiomyopathy experts. It has also been reviewed by external reviewers so it is a fairly collaborative study. It can be used at different levels. It may just help the clinician to suspect myocarditis. It may help a tertiary center to get equipped to help arrive at a diagnosis of myocarditis.

　　《国际循环》：非常感谢您接受《国际循环》的采访。第一个问题是关于今年7月份刚刚发布的心肌炎及心包疾病立场声明的，您认为该立场声明会对临床实践产生哪些影响？

　　Caforio教授：它将对临床实践产生非常好的影响，因为至少立场声明旨在为临床医生提供有关可疑心肌炎及心肌炎诊断及鉴别诊断的方向。该立场声明主要传达了临床心脏科医生便于理解的以下两方面的信息。其一是，综述了目前对疾病病因到治疗各方面知识的认识。其二是提出了对临床可疑心肌炎的新诊断标准。心肌炎的问题在于其存在较大的异质性，常与其他疾病具有相似的临床表现。一般情况下，其主要表现为假性心肌梗死伴或不伴有肌钙蛋白增加，冠状动脉正常；急性、亚急性及慢性心力衰竭表现（伴或不伴有心脏扩张）；还可表现为心律失常，轻则表现为心悸，严重时可发生晕厥甚至突发心源性猝死；其急性发病时可能会导致心源性休克，危及患者的生命。这是其主要的临床表现，但是心肌炎是一种排除诊断。因此，诊断心肌炎时需要首先排除冠状动脉疾病及出现心肌炎临床表现综合征的已知病因。心肌炎的诊断不能仅仅依靠临床表现，通过临床表现我们只能说怀疑患者得了心肌炎。需要对患者进行心电图检查，立场声明的文件中以表格的形式详细列出了可以选择的多种心电图检查方法。你可以做心电图、动态心电图或心电图负荷试验，还可通过超声心动图、心脏核磁共振或血管造影等无创及有创心脏成像方法观察心肌结构及功能的改变。结构改变可能是整体的也可能是节段性的。心肌炎时，患者的射血分数可能会正常。CMR可能提示心肌炎的组织特征。立场声明指出，心肌炎应符合以下诊断标准：心肌炎临床表现，1种或更多心电图或其他仪器检查结果。如果患者不伴有症状，其诊断通常较为偶然，需要更确切的证据以建立临床怀疑。这仍然只是临床可疑心肌炎，确诊需要排除冠状动脉疾病并进行心内膜心肌活检。心内膜心肌活检不仅需符合组织学标准，还要采取免疫组化特殊染色，并通过分子学方法查明感染源，才能区分不同类型的心肌炎。所有自身免疫调节性的心肌炎是没有感染源的。当然，也有很多感染性心肌炎，还有一小部分心肌炎患者既存在感染源又伴有免疫激活。幸运的是，既存在感染源又伴有免疫激活的心肌炎患者所占的比例非常小，临床上大部分患者可以分类为感染性心肌炎及自身免疫性心肌炎。接下来立场声明中对各种无创及有创检查在诊断心肌炎中的作用作出了推荐，其目的是获得心肌炎病因学诊断，因为自身免疫性心肌炎可选用免疫抑制剂安全地进行管理和治疗。而存在感染源时则不能应用免疫抑制剂，并需要根据感染源的不同选择适宜的治疗药物。该立场声明是非常全面的，是由整个欧洲心肌炎及心肌病研究领域的专家来共同编写，并经过了外部专家的评审，是各方面权威专家通力合作的产物，对临床实践的各个层次均非常有用，有助于帮助临床医生知道哪些情况应怀疑心肌炎，有助于帮助三级医院配备心肌炎诊断的相应设备。