Toussaint M, Planche C, Villain E, Kachaner J
Laboratoire d'Histologie et Embryologie, Faculté de Médecine Necker-Enfants Malades, Paris, France.
Virchows Arch A Pathol Anat Histopathol. 1987;412(1):27-9. doi: 10.1007/BF00750727.
Restrictive cardiomyopathy is usually related to fibrosis of the endocardium or to an infiltrative disorder. However, in few cases, it can be due to isolated pathology of the myocytes but such alterations are not well characterized. This paper reports the disease in two 7 year old patients. There was severe venous congestion and catheterisation revealed increased end diastolic pressure in the ventricles. Both pericardial and myocardial biopsies were performed, as the clinical and haemodynamic data were indistinguishable from constrictive pericarditis. The structure of the pericardium was normal. The endocardium was not thickened. The interstitium of the myocardial tissue was not increased. Electron microscopic examination revealed intracellular masses of disorganized myofilaments. These large deposits may have produced decrease compliance of the myocytes and of the ventricular walls.
限制性心肌病通常与心内膜纤维化或浸润性疾病有关。然而，在少数情况下，它可能是由于心肌细胞的孤立性病变，但这种改变尚未得到充分表征。本文报告了两名7岁患者的这种疾病。存在严重的静脉充血，导管检查显示心室舒张末期压力升高。由于临床和血流动力学数据与缩窄性心包炎无法区分，因此进行了心包和心肌活检。心包结构正常。心内膜未增厚。心肌组织的间质没有增加。电子显微镜检查显示细胞内有杂乱的肌丝团块。这些大的沉积物可能导致了心肌细胞和心室壁顺应性的降低。
