Koide T, Narita T, Sumino S
Br Heart J. 1982 May;47(5):507-10. doi: 10.1136/hrt.47.5.507.
A 49-year-old women with congestive heart failure and heart block died of cerebral embolism. Clinical and echocardiographic findings suggested a diagnosis of atypical dilated cardiomyopathy with predominantly right ventricular involvement. At necropsy, all the cardiac chambers were slightly dilated and the interventricular septum and the left ventricular wall were of normal thickness and symmetry. Histological examination, however, disclosed extensive disarray of abnormal myocardial tissue, especially in the interventricular septum. Her father had similar clinical and echocardiographic findings, while one of her brothers had typical hypertrophic cardiomyopathy at necropsy. It is likely that the patient actually had inherited hypertrophic cardiomyopathy. The case illustrates the difficulty in diagnosing hypertrophic cardiomyopathy when based solely on the left ventricular gross anatomy.
一名49岁患有充血性心力衰竭和心脏传导阻滞的女性死于脑栓塞。临床和超声心动图检查结果提示诊断为非典型扩张型心肌病,主要累及右心室。尸检时,所有心腔均轻度扩张,室间隔和左心室壁厚度及对称性正常。然而,组织学检查发现广泛的异常心肌组织排列紊乱,尤其是在室间隔。她的父亲有类似的临床和超声心动图表现,而她的一个兄弟尸检时患有典型的肥厚型心肌病。该患者很可能实际患有遗传性肥厚型心肌病。该病例说明了仅根据左心室大体解剖结构诊断肥厚型心肌病的困难。