单纯心壁病损性扩张心脏的病理基础分析

目的观察单纯心壁病损造成心脏扩张者心脏的病理形态特征,为临床和影像提供鉴别诊断参考依据。方法2004年6月至2006年6月底,对阜外心血管病医院进行心脏移植的56例受体心脏在离体后立即进行了肉眼观察、测量和摄影记录,并进行了全面的病理组织学观察。以其中单纯心壁病损造成的心脏扩张者进行临床病理对比分析,观察其形态特点。结果56例受体心脏中38例为单纯心壁病损造成的心脏扩张者,占67．9％。这38例中50．0％为原发性扩张型心肌病,23．7％为致心律失常性右心室心肌病,15．8％为缺血性心肌病,其余的10．5％为局灶性心肌致密化不全、巨细胞性心肌炎和特异性心肌病中的酒精性心肌病和高血压性心肌病。38例中临床诊断与病理诊断不相同的有15例,不相符率为39．5％,不相符率较高的依次为巨细胞性心肌炎（100％）、缺血性心肌病（83．3％）和致心律失常性右心室心肌病（77．8％）。不相符的这几种疾病的原临床和影像学诊断都是原发性扩张型心肌病。致心律失常性右心室心肌病、缺血性心肌病、心肌致密化不全和巨细胞性心肌炎都有特征性的病理形态表现,酒精性心肌病和高血压性心肌病等的病理诊断需要参考临床资料。结论进行心脏移植病例受体心脏的病理学检查有利于提高心脏病的临床和影像诊断的正确率,有扩张表现的心脏病的诊断要排除心脏扩张的继发原因,并要注意与终末期心脏疾病作鉴别。

Pathological characteristics of dilated hearts with sole myocardial wall damage in recipients

OBJECTIVE: It is difficult to differentiate the causes of dilated cardiomyopathy only by clinical evaluation and image analysis. Pathomorphologic examinations on diseased hearts may help to improve the diagnosis accuracy. METHODS: Fifty-six extransplanted hearts from June, 2004 to June, 2006 were examined. Gross and histopathological findings were recorded, photographed and final pathological diagnosis was compared to clinical diagnosis. RESULTS: Dilations were caused by sole myocardial wall damage in 38 (67.9%) of the 56 patients, including 19 primary dilated cardiamyopathy, 9 arrhythmogenic right ventricular cardiomyopathy, 1 non-compaction cardiomyopathy, 6 ischemic cardiomyopathy, 1 alcoholic cardiomyopathy, 1 hypertensive cardiomyopathy and 1 giant cell myocarditis. The clinical and pathological diagnoses were different in 15 cases (39.5%). The most discrepancies were arrythmogenic right ventricular cardiomyopathy (77.8%), ischemic cardiomyopathy (83.3%), and giant cell myocarditis (100%). CONCLUSIONS: This pathological study of recipient hearts showed a high portion of patients with arrhythmogenic right ventricular cardiomyopathy and ischemic cardiomyopathy were misdiagnosed as primary cardiomyopathy. Correct diagnosis of primary cardiomyopathy needs to rule out possible secondary causes of myocardial dilation.