Child J S, Perloff J K
UCLA School of Medicine.
Cardiol Clin. 1988 May;6(2):289-316.
In parallel with the rapidly developing interest in the diastolic properties of ventricular function in the 1970s, the restrictive cardiomyopathies have taken their place as the third major category of primary heart muscle disease. The restrictive cardiomyopathies are characterized by primary abnormalities of diastolic ventricular function with normal to near normal systolic performance and little or no increase in end-diastolic or end-systolic dimensions of either right or left ventricle. The restrictive abnormality of ventricular function can result from myocardial or endomyocardial disease, the etiologies of which may be known or unknown. Diastolic dysfunction that is essentially myocardial can be idiopathic (probably an enzymatic/metabolic disturbance), infiltrative (myocardial interstitium), or within myocardial cells (storage diseases). Diastolic dysfunction that results from endomyocardial disease is typified by endomyocardial fibrosis or the hypereosinophilic syndrome, although carcinoid, metastatic malignancies, radiation, and anthracycline toxicity may be accompanied by endomyocardial restriction. Echocardiography and Doppler ultrasound, MRI, and radionuclear techniques have been major advances in providing diagnostic precision, contributing materially to the clinical identification of the restrictive cardiomyopathies as well as discriminating certain of their etiologies.
20世纪70年代，随着人们对心室功能舒张特性的兴趣迅速增长，限制性心肌病已成为原发性心肌疾病的第三大主要类型。限制性心肌病的特征是舒张期心室功能原发性异常，收缩功能正常或接近正常，右心室或左心室舒张末期或收缩末期内径几乎没有增加或增加很少。心室功能的限制性异常可由心肌或心内膜疾病引起，其病因可能已知或未知。本质上是心肌性的舒张功能障碍可以是特发性的（可能是酶/代谢紊乱）、浸润性的（心肌间质）或心肌细胞内的（贮积病）。心内膜疾病导致的舒张功能障碍以内膜心肌纤维化或高嗜酸性粒细胞综合征为典型，尽管类癌、转移性恶性肿瘤、放射和蒽环类药物毒性可能伴有心内膜限制。超声心动图、多普勒超声、磁共振成像和放射性核素技术在提高诊断准确性方面取得了重大进展，对限制性心肌病的临床识别以及区分某些病因有很大帮助。
