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.%By far,there has yet been well-acknowledged diagnostic criterion for restrictive cardiomyopathy (RCM) ,a group of diseases characterized of restrictive diastolic dysfunction. This warranted comprehensive measures, including assessment of clinical manifestations and imaging as well as eehocardiography and cardiac magnetic resonance being critical adjuvant appraisals. RCM may be mostly secondary to systemic diseases,for instance,amyloidosis, hemosiderosis and hypereosinophilic syndrome. Additionally, idiopathic RCM could have arisen from myosin or desmin gene mutation. The currently poor prognosis of RCM may be related to the lack of effective medications available. Cardiac transplantation at early stages might be helpful to improve prognosis in a subset of patients.
展开▼
