法洛四联症右心室流出道心肌的超微结构及力学特性  

Myocardium characters of right ventricular outflow tract in Tetralogy of Fallot

作　　者：李晓峰[1] 李仲智[1] 郎志奇[2] 刘志诚[3] 李海云[3] 郝冬梅[3] 

机构地区：[1]首都医科大学附属北京儿童医院心外科,100045 [2]首都医科大学附属北京儿童医院电镜室,100045 [3]首都医科大学生物医学工程系

出　　处：《中华小儿外科杂志》2000年第5期268-271,共4页Chinese Journal of Pediatric Surgery

摘　　要：目的　研究法洛四联症 (TOF)右心室流出道心肌细胞的超微结构变化及心肌组织的生物力学性能。方法　对 2 4例TOF根治术手术前后的右心室及肺动脉的压力变化进行对比研究。对其中 7例TOF与对照组 7例先心病小室缺、小房缺应用透射电镜进行右心室流出道心肌的超微结构研究。对 4例TOF与 2例非先心病死亡右心室心肌进行多样本量的对比 ,采用计算机控制的小力学量软组织力学测试平台进行右心室心肌力学的研究。结果　TOF右心室流出道的心肌组织存在着很多超微结构异常的心肌细胞。TOF右心室流出道心肌组织的力学特点表现为过软 ,而右心室外壁表现为过硬 ,TOF根治术经过较为彻底地切除肥厚肌束 ,没有残余右心室流出道梗阻存在 ,肺动脉瓣前后无阶差存在 ,P >0 .0 5。 2 4例TOF无手术死亡。结论　建议TOF根治中 ,在疏通右心室流出道、切除肥厚肌束时不必过分考虑其功能作用。Objective To study the ultrastructural changes and biomechanical characteristics of myocardium of the right ventricular outflow tract in tetralogy of Fallot (TOF). Methods The preoperative and postoperative pressures of AO,RV,PA of 24 consecutive patients with TOF were studied. Seven age matched patients with small ASD or VSD were used as the control group in electro microscopy (JEM 1200) examination. Two non CHD related myocardial specimens were used as the control for mechanical analysis. Results The right ventricular outflow tract in TOF contained more abnormal myocytes. Unlike the control group, myocardium of right ventricular wall in TOF was more rigid. On the other hand, myocardium of right ventricular tract was biomechanically softer than the control group. This led to reduced voluntary contraction of right outflow tract and reduced response to passive stretching. All TOF patients underwent primary one stage repair in the previous year. The result was satisfactory and there was no early mortality or residual RVOTO. Conclusion For total repair of TOF, we suggest complete resection of hypertrophied muscles in right ventricular outflow tract.

关 键 词：法洛四联症 心肌 右心室流出道 超微结构 

分 类 号：R725.4[医药卫生—儿科]