38例限制型心肌病的临床回顾性分析——附2例心脏移植报告.docVIP

38例限制型心肌病的临床回顾性分析 ——附2例心脏移植报告 董太明 徐名伟* 黄新胜 靳立军 张斌 吴书林 （广东省心血管病研究所心血管内科 广东 广州市510100 * 广东省揭阳市人民医院内科） 摘要 [目的] 评价超声心动图（UCG）、核磁共振成像（MRI），心血管造影（ACG）、心内膜心肌活检（EMB）和剖胸探查术对限制型心肌病（RCM）的诊断价值，总结诊断RCM的临床思维方式及诊断程序,并讨论RCM的临床治疗。[方法] 对有不明原因的心力衰竭和（或）心室压塞等临床表现为RCM的38例患者施行UCG、MRI、ACG、EMB、剖胸探查术等检查并进行综合临床分析，对2例心脏移植疗效进行评估。[结果] UCG、MRI、ACG、EMB和剖胸探查术对获得RCM诊断的病例比例分别是28/38、5/11、6/7、3/3、2/2，2例患者接受心脏移植手术治疗，手术均成功，1例随访4年6个月，术后心功能明显改善，并能恢复轻～中度工作；另1例15个月后死于心脏排斥反应和肝坏死。[结论] UCG、MRI、ACG、EMB和剖胸探查术对RCM的诊断具有重要价值，但阳性率不同，须综合应用才能更好提高诊断水平;RCM诊断程序依次为UCG和MRI，如有疑问行ACG及EMB，仍有疑问，可考虑施行剖胸探查术。外科心脏移植治疗可能是对处于晚期RCM病人的最佳选择。 [关键词] 心肌病 限制型 诊断 鉴别诊断 治疗 中图分类号： 文献标识码：A The retrospective study of 38 cases of idiopathic restrictive cardiomyopathy Dong Taiming, Xu Minwei, Huang Xingsheng, Jin Lijun, Zhang Bin, Wu Shulin.Cardiology Department, Guangdong Provincial Cardiovascular Institute. 96 Road Dongchun, Guangzhou P.R.China 510100 Key words Cardiomyopathy; Restrictive; Diagnosis; Treatment [Abstract] To evaluate the diagnostic value of ultrasonic cardiography (UCG),magnetic resonance image(MRI), angiocardiography; （ACG） and endomyocardiol biopsy（EMB） for restrictive cardiomyopthy, to explore the clinical approach and diagnostic process for RCM diagnosis. Methods This was a restropective study of patients with unknown cause heart failure at Guangdong Provincial Cardiovascular Institute between March 1992 and June 2004. Thirty eight patients with mean age 31.6?5.6 years old(1-76 years old) met diagnostic criteria for RC平M. All patients were examined by UCG. MRI, ACG and EMB were performed for differentiation of other heart disease in some of patients. Two patients underwent thoracotomy eventually, two patients had cardiac transplantation. Results 28 of 38 cases were diagnostic of RCM by UCG. only 5 of 11 cases were correctly diagnosed as RCM by MRI, but the others were misdiagnosed as CP by MRI. 6 of 7 RCM patients got correct diagnosis with ACG, pathologic