An autopsy case of cardiomyopathy with restrictive physiology in a child.

Heart and vessels. Supplement Pub Date : 1990-01-01
M Ishijima, S Kawai, R Okada, T Ino, K Yobuta
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Abstract

An autopsy case of cardiomyopathy with restrictive physiology associated with subendocardial myocardial necrosis and fibrosis in a seven-year-old child is described. Cardiac catheterization showed high left ventricular end-diastolic pressure with a dip-and-plateau pattern. Macroscopically, marked dilatation with fibroelastosis in both atria and mild dilatation in both ventricles were observed. Histologic examination revealed extensive subendocardial necrosis with marked disorganization of myocardial cells and moderate arteriolosclerosis. We speculate that these extensive myocardial lesions, which contributed to the restrictive hemodynamic changes, could have been induced by anoxia or a Ca2+ transport abnormality of unknown cause.

儿童心肌病伴限制性生理解剖一例。
解剖心肌病与限制性生理相关的心内膜下心肌坏死和纤维化在一个7岁的孩子被描述。心导管检查显示左室舒张末期压高,呈降平型。在宏观上,观察到两心房明显的扩张和纤维弹性增生,两心室轻度扩张。组织学检查显示广泛的心内膜下坏死,心肌细胞明显紊乱,微动脉中度硬化。我们推测,这些广泛的心肌病变导致限制性血流动力学改变,可能是由缺氧或未知原因的Ca2+转运异常引起的。
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