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限制型心肌病(RCM)是一类以限制性舒张功能障碍为主要特征的心肌病,其诊断目前尚缺乏公认的标准,需要结合临床表现和影像学检查综合诊断,超声心动图和心脏磁共振(CMR)是重要的辅助检查。RCM多继发于全身疾病,常见于淀粉样变性、含铁血黄素沉积、高嗜酸细胞综合征等;一些肌结蛋白或结蛋白基因突变可导致特发性RCM。RCM预后较差,目前尚缺乏有效的药物治疗手段。对于一些患者,早期进行心脏移植可能会改善其预后。
Restrictive cardiomyopathy (RCM) is a class of cardiomyopathy characterized by restrictive diastolic dysfunction. There is currently no recognized standard for its diagnosis, which requires a combination of clinical manifestations and imaging diagnosis, echocardiography and cardiac magnetic resonance (CMR) is an important auxiliary examination. RCM secondary to systemic diseases, common in amyloidosis, hemosiderin deposition, hypereosinophilic syndrome, etc .; some of myosin or desmin gene mutations can lead to idiopathic RCM. RCM poor prognosis, the current lack of effective drug treatment. For some patients, early heart transplantation may improve its prognosis.