法乐氏四联症心室超微结构的特征及其临床意义(英文)

The Ultrastructural Characteristics of the Ventricular Myocytes in Tetralogy of Fallot and Its Clinical Implications

探讨四联症心肌细胞超微结构改变的特征及其临床意义。作者对14例一期根治的法氏四联症在转流开始时行左右心室肌活检。光镜显示左室心肌细胞短径为13．4±3μm，右室为17．8±4μm，左右心室的高尔基体及肌浆网发育良好，核糖体数目增加，线粒体积聚，核高度畸变。在左右心室均存在肌原纤维自溶、肌节缩短、排列紊乱及T管系统扩张，然而肌节缩短和T管扩张程度左室均甚于右室。四联症心肌细胞超微结构改变表明由多种原因造成心肌细胞广泛损害。早期根治可能减轻低心排综合症的发生率以及改善远期心功能。

We examined the ultrastructure of right and left ventricular myocardial biopsies obtained prior to cardiopulmonary bypass (CPB) in 14 cases undergoing primary total correction of tetralogy of Fallot. The diameter of myocytes was 13.4 ±3μm in left ventricle and 17.8±4μm in right ventricle. In both right and lelt ventricles the Golgi apparatus and rough endoplasmic reticulum (RER) developed well, the numbers of ribosome increased, mitochondria accumulated, nucleus highly deformed. There were lysis of mpofibril, shortage and disarrangement of sarcomere, dilation of T- tubule System in both ventricles. However the degree of shortage of sarcomere and dilatation of T-tubule system in left ventricle were more serious than that in right ventricle. The alterations of ultrastructure of myocytes in Tetralogy of Fallot indicate there were widespread damage resulted from multiple lactors. The early ropair may decrease the occurrence of low output syndrome and better remote cardiac function.