The spectrum of dilated cardiomyopathy - The Johns Hopkins experience with 1,278 patients
The spectrum of dilated cardiomyopathy - The Johns Hopkins experience with 1,278 patients
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DOI：
10.1097/00005792-199907000-00005
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发表时间：
1999-07-01
期刊：
影响因子：
1.6
通讯作者：
Kasper, EK
中科院分区：
文献类型：
--
作者：
Felker, GM;Hu, WM;Kasper, EK
扩张型心肌病，定义为左心室收缩功能障碍伴心室扩张，是许多病理性心脏过程的最终共同途径。扩张型心肌病的潜在病因很多。至少有75种特定的心肌疾病可导致左心室收缩功能障碍，伴或不伴充血性心力衰竭的临床综合征（67）。虽然扩张型心肌病的病因有多种，但仍有相当一部分病例病因不明（31）。虽然肌内膜活检有助于确定某些患者扩张型心肌病的病因，但确定特定患者病因的治疗和预后效用尚不清楚（38，40）。我们报告了1982年12月29日至1998年9月1日期间，约翰霍普金斯心肌病服务中心收治的1,278例心肌病患者的诊断评价结果。本研究的目的是通过一个大的转诊系列来描述心肌病的病因，并确定特定的临床情况，在这些情况下，肌内膜活检可能对这些患者的诊断评估有用。在这些病例中，有672例先前已报告（31）。
Dilated cardiomyopathy, defined as left ventricular systolic dysfunction with chamber dilation, represents a final common pathway for many pathologic cardiac processes. The potential etiologies of dilated cardiomyopathy are numerous. At least 75 specific diseases of heart muscle can result in left ventricular systolic dysfunction, with or without the clinical syndrome of congestive heartfailure (67). Although multiple causes of dilated cardiomyopathy have been described, no clear etiology can beidentified in a substantial percentage of cases (31). While endomyocardial biopsy can be helpful in defining the etiology of dilated cardiomyopathy in some patients, the therapeutic and prognostic utility ofidentifying a specific cause for a given patient remains unclear (38, 40). We report the results of the diagnostic evaluation of a series of 1,278 patients with cardiomyopathy referred to the Johns Hopkins Cardiomyopathy Service between December 29, 1982, and September 1, 1998. The aims of this study are to describe the etiology of cardiomyopathy in a large referral series and to identify specific clinical situations in which endomyocardial biopsy may be usefulin the diagnostic evaluation of these patients. Of these cases, 672 have been reported previously (31).