Ishijima M, Kawai S, Okada R, Ino T, Yobuta K
Research Laboratory for Cardiovascular Pathology, Tokyo, Japan.
Heart Vessels Suppl. 1990;5:70-3.
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.
本文描述了一名七岁儿童患限制性心肌病伴心内膜下心肌坏死和纤维化的尸检病例。心脏导管检查显示左心室舒张末期压力升高，呈下陷-高原波型。大体检查可见心房显著扩张伴纤维弹性组织增生，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，小动脉硬化中度。我们推测，这些导致限制性血流动力学改变的广泛心肌病变可能是由缺氧或原因不明的钙离子转运异常所致。
