Hufnagel G, Pankuweit S, Maisch B
Abteilung Innere Medizin-Schwerpunkt Kardiologie, Philipps-Universität Marburg.
Med Klin (Munich). 1998 Apr 15;93(4):240-51. doi: 10.1007/BF03044800.
Diagnosis of inflammatory dilated cardiomyopathy relies on the histological and immunohistological examination of endomyocardial biopsies. Only with the demonstration of the etiological agents in the myocardium specific therapy can be attempted. Whereas the spontaneous course of endemic myocarditis with little hemodynamic impairment is fair, the prognosis of symptomatic myocarditis and dilated cardiomyopathy is poor, with complete restitution in 35% and a 10-year survival rate of 30%. Restriction of physical activity is a validated form of therapy with normalization of the heart size in 40 to 60%. Symptomatic medical therapy consists of digitalis, diuretics, ACE-inhibitors and vasodilators and betablocker therapy, where a reduction of mortality was demonstrated in clinical (sub)studies up to 60%. Specific forms of therapy in inflammatory cardiomyopathy rely on the demonstration or lack of viral persistence or signs of autoreactivity in the myocardial tissue. Immunosuppressive therapy in autoimmune forms improved cardiac function in up to 60% of the patients in controlled trials, when compared to controls (40%). The double-blind randomized myocarditis treatment trial, which unfortunately did not distinguish viral from autoimmune myocarditis could not demonstrate such a benefit, however. Depending on the etiology of the disease, immunomodulation with immunoglobulins or interferon or antiviral therapy with hyperimmunoglobulins are presently tested in clinical treatment trials (ESETCID) in patients with enterovirus-positive or cytomegalovirus-positive and adenovirus-positive chronic myocarditis. Specific therapies are aimed to avoid the progression of the disease which may ultimately lead to heart failure with a cardiac assist device or heart transplantation as ultimate therapeutic option.
炎症性扩张型心肌病的诊断依赖于心内膜心肌活检的组织学和免疫组织学检查。只有在心肌中证实有致病因子后,才能尝试进行特异性治疗。地方性心肌炎的自然病程中血流动力学损害较小,预后尚可,而有症状的心肌炎和扩张型心肌病预后较差,35%可完全恢复,10年生存率为30%。限制体力活动是一种有效的治疗方式,40%至60%的患者心脏大小可恢复正常。对症药物治疗包括使用洋地黄、利尿剂、血管紧张素转换酶抑制剂、血管扩张剂和β受体阻滞剂,临床(亚)研究表明这些药物可使死亡率降低达60%。炎症性心肌病的特异性治疗方式取决于心肌组织中是否存在病毒持续感染或自身反应迹象。在对照试验中,与对照组(40%)相比,自身免疫型患者采用免疫抑制治疗后,高达60%的患者心脏功能得到改善。然而,不幸的是,双盲随机心肌炎治疗试验未区分病毒性心肌炎和自身免疫性心肌炎,未能证明有此类益处。根据疾病的病因,目前正在对肠道病毒阳性、巨细胞病毒阳性和腺病毒阳性的慢性心肌炎患者进行免疫球蛋白或干扰素免疫调节治疗或高免疫球蛋白抗病毒治疗的临床治疗试验(ESETCID)。特异性治疗旨在避免疾病进展,疾病最终可能导致心力衰竭,此时心脏辅助装置或心脏移植是最终的治疗选择。