埃勃斯坦(Ebstein)氏畸形是一种少见的先天性心脏病,文献报道较少。我们曾收治一例巨大心脏患者,临床诊断为“原发性心肌病”、“克山病”,经尸解证实为Ebstein氏畸形。患者男性,41岁。因劳动时心慌、气促、乏力5年,加重伴夜间不能平卧10天而 Ebstein’s deformity is a rare congenital heart disease, reported in the literature is less. We have treated a huge cardiac patient, the clinical diagnosis of “primary cardiomyopathy”, “Keshan disease” confirmed by the autopsy Ebstein’s deformity. Male patient, 41 years old. Due to labor, palpitation, shortness of breath, fatigue for 5 years, aggravated with the night can not lie 10 days