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[ESC2013]欧洲心肌炎和心包疾病2013立场声明解读——Alida L.p. Caforio教授访谈

作者:  A.L.P.Caforio   日期:2013/9/13 14:08:53

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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。

  <International Circulation>: You mentioned that the biopsy is the gold standard of diagnosis. A lot of hospitals will not have the capacity to perform endomyocardial biopsies, so what should doctors do in those situations?

  Prof Caforio: This is the first level or foundation for building up protocols for diagnosis. At the moment, not all hospitals have endomyocardial biopsy facilities so the document includes indications for clinically identifying those patients who are at higher risk and who should be sent to a tertiary center with biopsy expertise as well as expertise in mechanical assistance and heart transplantation. There is a subset of generally young patients with very severe disease who can be rescued mainly by immune suppression but the condition can progress very rapidly so time should not be wasted with arriving at a diagnosis. The patient should be sent as soon as possible to a place where there is CMR and biopsy expertise. Without previous studies, the problem with myocarditis is that immunosuppression as a first-line treatment, although it is actually quite safe in an unselected population because the majority of myocarditis patients in the experience of the major centers have an autoimmune-mediated component and that will not always be disastrous, it is important to initiate this therapy only after the exclusion of active infection. Blind immune suppression is not indicated.

  《国际循环》:您曾提到心肌活检是诊断心肌炎的金标准,但很多医院不具备进行心肌活检的能力,在这种情况下医生应如何做?

  Caforio教授:目前,并不是所有医院都具备心肌活检相关设备。这是心肌炎诊断面临的首要难题。因此,立场声明文件推荐那些高危患者到有心肌活检经验和机械支持、心脏移植能力的三级医院进行心肌活检。很多伴有严重疾病的年轻患者可以通过应用免疫抑制剂获救,但是,疾病的进展是非常快的,因此不能浪费时间,一定要尽早确诊。因此,当无法行心肌活检时,应尽快将患者转诊至能进行心脏核磁共振及心肌活检的医院。使用免疫抑制剂必须注意安全性。由于缺乏研究,尽管免疫抑制剂在未经选择的患者中应用实际上是非常安全的,因为到大的中心就诊的大部分心肌炎患者都为自身免疫性,使用免疫抑制剂并不是灾难性的。但我们仍应记住只有在排除患者处于感染活动期后才能应用免疫抑制剂。盲目应用免疫抑制剂是被禁止的。

  <International Circulation>: The prognosis for myocarditis is variable depending on etiology. What are the useful tools for doctors to assess the prognosis of patients?

  Prof Caforio: This is a very important question. There are several papers in the literature but very few. For prognosis you should only look at the papers where there is confirmation of diagnosis by biopsy. In these papers it is clear that systolic dysfunction(left and right) at presentation is a negative prognostic factor as well as the presence of giant cell myocarditis or eosinophilic myocarditis. Left and/or right ventricular dysfunction and all the conventional markers of heart failure are relevant in determining prognosis including dilatation of the left or right ventricles but these are not enough. The risk stratification is not accurate because some acute conditions caused by myocarditis can actually regress such as some viral forms. It is important for the clinician to know the prognostic factors clinically to help identify those at higher risk but we need more multi-center control data to refine our risk stratification. One of the aims of the paper is also to have shared criteria for clinically suspected and biopsy-proven myocarditis and to set up international registries to see in these two conditions what the main prognostic factors are. The third and most important aim is to be able to set up multi-center randomized trials on etiology-specific therapies in patients that are recruited according to their etiology. This will give insights into what is the best therapeutic strategy according to the different forms of disease.

  《国际循环》:心肌炎的预后在一定程度上是与其病因有关的,临床医生应如何评估患者的预后?

  Caforio教授:是的,这是个非常重要的问题。目前的相关文献较少,仅有几篇相关文章。为了解预后,我们只能参考那些经心肌活检确诊的心肌炎相关研究。在这些文章中,很明确如果患者存在左室及右室收缩功能障碍,或为巨细胞性或嗜酸性细胞性心肌炎,预后通常较差。左室及右室功能障碍及心力衰竭严重程度常规标志物如左室和右室扩大程度与预后相关,但是仅仅评估这些是不够的。因为心肌炎所引发的急性变化可以缓解,如某些病毒性心肌炎,造成危险分层不够准确。识别临床预后因素对临床医生很重要,有助于识别高危患者。我们需要更多的多中心研究数据来细化心肌炎患者的危险分层。立场声明的一个目的就是制定临床可疑心肌炎及活检证实的心肌炎的诊断标准,并建立国际注册研究以确定在这两种状态下,影响预后的主要因素。第三个目的也是最重要的目的在于开展针对病因特异性治疗的多中心临床试验,这些试验将根据病因学纳入患者以探索不同病原学心肌炎的最佳治疗策略。

  <International Circulation>: With respect to therapy, what is your opinion on ICD and CIT for treatment?

  Prof Caforio: This is another very important topic and very controversial also in that there is not enough data. We have recommendations for ICD and arrhythmia as well. The recommendation is that in biopsy-proven myocarditis (you have to start from a stable confirmed diagnosis) it depends whether you are dealing with sarcoidosis or giant cell myocarditis or just with lymphocytic myocarditis, some forms of myocarditis improve spontaneously. Arrhythmia may be present in the acute phase but won’t be present in the follow-up. In these cases, the recommendation is in keeping with the international guidelines, is to, if possible, defer ICD implantation in the acute phase of myocarditis and if you feel that there is a high arrhythmia risk you can consider protecting the patient with a life vest for a few months and get confirmation of activity by biopsy to direct your strategy. In some very severe forms of myocarditis however, such as sarcoidosis and giant cell myocarditis, in the stable (not hyperacute) phase, there may be greater indication for ICD placement because they are both highly arrhythmogenic conditions that may be stabilized by immune suppression even before device implantation but can always have a relapse. Again, etiology dictates therapeutic management.

  《国际循环》:就心肌炎的治疗而言, 您对ICD和CIT有什么看法?

  Caforio教授:这是另外一个心肌炎治疗的重要话题,目前尚存争议,相关证据尚不充分。我们对ICD和心律失常有相关推荐:在心肌活检确诊的心肌炎患者中(我们必须从建立一项稳定确切的诊断开始),是否植入ICD取决于是否为肉瘤样病、巨细胞性心肌炎还是仅仅为淋巴细胞性心肌炎或一些自限性病变。心律失常可能会在急性期出现,随访时消失。在这种状况下,推荐是与国际指南保持一致,那就是在心肌炎的急性期尽可能地推迟行ICD植入术。如果患者存在较高的心律失常风险,你可以考虑对患者采取几个月的生命保护措施,在心肌活检后,根据结果指导治疗策略。特别严重的心肌炎,如肉瘤样病和巨细胞性心肌炎,在稳定期(非超急期)时,则更多地选用植入ICD。因为尽管ICD植入前免疫抑制剂可稳定病情,但这些疾病高度致心律失常,病情稳定后可复发。一旦反复意味着心室功能恶化及较高的心律失常发生风险。也就是说,病因决定心肌炎的治疗与管理。

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