TIAN Zhuang, ZENG Yong, FANG Quan, ZHAO Da-chun, CUI Quan-cai, CHENG Kang-an, FANG Li-gang, GAO Peng, CHENG Zhong-wei, JIANG Xiu-chun, SUN Rong-liang
Department of Cardiology, Peking Union Medical College Hospital, Chinese Academy of Medical Science, Peking Union Medical College, Beijing 100730, China.
Zhonghua Xin Xue Guan Bing Za Zhi. 2010 Sep;38(9):786-9.
restrictive cardiomyopathy (RCM) is characterized by impairment of ventricular filling during diastole with preserved systolic function. The clinical and histopathological profile on endomyocardial biopsy of 25 consecutive patients with RCM was analyzed in this study.
twenty-five patients with diagnosis of RCM and underwent endomyocardial biopsy (EMB) were enrolled in the study. The clinical characteristics, electrocardiogram, serum chemistry, right heart catheter and cardiac pathology results were obtained.
heart failure symptom was present in all 25 patients and left ventricular size and function were normal or near normal while serum brain natriuretic peptide (577 pg/ml) was moderately elevated. Right atrial and ventricular end-diastolic as well as pulmonary capillary wedge pressures derived from right heart catheter examination were increased. Amyloid deposition were evidenced in 16 and eosinophilic myocarditis in 2 patients upon pathological examination of EMB. In the remaining 7 patients, 3 were diagnosed idiopathic RCM, 2 were diagnosed as amyloidosis by biopsy from non-cardiac tissue and etiology remained unknown in 2 patients. Thus, conclusive diagnosis was made on EMB samples in 84% (21/25) patients of RCM.
RCM may result from various local and systemic disorders. EMB is helpful for identifying the underlying etiology.
限制型心肌病(RCM)的特征是舒张期心室充盈受损而收缩功能保留。本研究分析了连续25例RCM患者心内膜心肌活检的临床和组织病理学特征。
本研究纳入25例诊断为RCM并接受心内膜心肌活检(EMB)的患者。获取其临床特征、心电图、血清化学指标、右心导管检查结果及心脏病理学结果。
25例患者均有心力衰竭症状,左心室大小和功能正常或接近正常,而血清脑钠肽(577 pg/ml)中度升高。右心导管检查测得的右心房和心室舒张末期以及肺毛细血管楔压升高。心内膜心肌活检病理检查显示16例有淀粉样蛋白沉积,2例有嗜酸性粒细胞性心肌炎。其余7例患者中,3例诊断为特发性RCM,2例经非心脏组织活检诊断为淀粉样变性,2例病因不明。因此,84%(21/25)的RCM患者通过心内膜心肌活检样本做出了明确诊断。
RCM可能由多种局部和全身性疾病引起。心内膜心肌活检有助于确定潜在病因。