Sigusch H H, Reinhardt D, Figulla H R
Klinik für Innere Medizin III, Kardiologie-Angiologie-Intensivmedizin, Klinikum der Universität Jena.
Med Klin (Munich). 1998 Apr 15;93(4):236-9. doi: 10.1007/BF03044799.
The main feature of idiopathic dilated cardiomyopathy is the dilation and impaired contractility of the left ventricle or both ventricles. The clinical picture with forward and backward failure is based on the pump impairment of the left ventricle. However, the clinical presentation of patients with dilated cardiomyopathy is indistinguishable from any other secondary form of heart failure. The symptoms of myocarditis are also often determined by the degree of left ventricular dysfunction and--apart from perimyocarditis-associated precordial discomfort--therefore also often indistinguishable from dilated cardiomyopathy. The differentiation of dilated cardiomyopathy from other myocardial diseases by noninvasive methods is insufficient. Without invasive tests about 1/3 of the patients will be diagnosed incorrectly. Therefore, invasive diagnostics including coronary angiography are necessary to differentiate dilated cardiomyopathy from other diseases, especially coronary artery disease. Standard laboratory findings and cytokine serum concentrations (e.g. TNF-alpha) are not suitable to differentiate dilated cardiomyopathy and myocarditis and endomyocardial biopsy is indicated. Endomyocardial biopsies have to undergo evaluation by standard histology and immunohistology, and should be tested for the persistence of infectious agents. According to cardiac catheterization and evaluation of the endomyocardial biopsy idiopathic left ventricular dysfunction can be further stratified using the criterion of a myocardial virus persistence and the presence/absence of inflammatory infiltrates. Idiopathic dilated cardiomyopathy (approximately 70 to 75%), virus-associated dilated cardiomyopathy (approximately 20 to 25%), myocarditis (approximately 7%) and autoimmune myocarditis (approximately 3%) are the 4 possible resulting forms of idiopathic left ventricular dysfunction. Beside conventional medical therapy there are new therapeutic concepts e.g. using interferon for enterovirus-positive patients and immunosuppression for autoimmune, virus-negative patients with a cellular infiltrate.
特发性扩张型心肌病的主要特征是左心室或双心室扩张及收缩功能受损。左心室泵功能障碍导致了心力衰竭的临床表现，既有前向性衰竭也有后向性衰竭。然而，扩张型心肌病患者的临床表现与任何其他继发性心力衰竭形式并无差异。心肌炎的症状通常也取决于左心室功能障碍的程度，并且——除了与心包心肌炎相关的心前区不适外——因此也常常与扩张型心肌病难以区分。通过非侵入性方法区分扩张型心肌病与其他心肌疾病并不充分。如果不进行侵入性检查，约三分之一的患者会被误诊。因此，包括冠状动脉造影在内的侵入性诊断对于区分扩张型心肌病与其他疾病，尤其是冠状动脉疾病是必要的。标准实验室检查结果和细胞因子血清浓度（如肿瘤坏死因子-α）不适用于区分扩张型心肌病和心肌炎，此时心内膜活检是必要的。心内膜活检必须通过标准组织学和免疫组织学进行评估，并且应该检测是否存在感染因子。根据心导管检查和心内膜活检的评估结果，可依据心肌病毒持续存在的标准以及炎症浸润的有无，对特发性左心室功能障碍进行进一步分层。特发性扩张型心肌病（约70%至75%）、病毒相关性扩张型心肌病（约20%至25%）、心肌炎（约7%）和自身免疫性心肌炎（约3%）是特发性左心室功能障碍的4种可能结果形式。除了传统的药物治疗外，还有一些新的治疗理念，例如对肠道病毒阳性患者使用干扰素，对有细胞浸润的自身免疫性、病毒阴性患者进行免疫抑制治疗。
