心肌致密化不全的超声心动图与病理检查对照研究

目的 探讨心肌致密化不全(NVM)的超声心动图表现与病理特征，以期尽早诊断并预防致死性心律失常和栓塞发生。方法 超声心动图检查方法以四腔观、左心室长轴二腔观和左心室短轴观为主进行多切面扫查，重点观察近心尖1／3的左心室心肌与心内膜。结果 24例患者超声心动图检查均可见受累心腔内异常隆突、粗大的肌小梁和其间深陷的隐窝影像，其中3例合并其他先天性心脏畸形，20例伴有充血性心力衰竭症状，仅2例无任何症状。24例中13例行冠状动脉造影未见异常，2例因严重充血性心力衰竭而最终进行心脏移植。对受体心脏进行了病理解剖和组织学检查，受累心腔内显示有异常粗大的肌小梁和交错深陷的隐窝，从心底到心尖致密心肌逐渐异常变薄；心内膜为增厚的纤维组织，内层非致密心肌肌束粗大紊乱、细胞核异形，外层致密心肌肌束走行及形态基本正常，细胞核大小均匀。结论 心肌致密化不全具有特征性超声心动图改变和特有的病理组织学变化，可区别于原发性扩张型心肌病。超声心动图是诊断心肌致密化不全的重要手段。

Comparative study between echocardiographic manifestations and pathological examinations in noncompaction of ventricular myocardium

Objective By exploring echocardiographic ch aracters and pathologic examination of noncompacted ventricular myocardium(NVM), to prevent life-threatening arrhythmias and embolism. Methods Echocardiographic examinations included four-chamber view, two-chamber view and short axis view of left ventricle, with an emphasis on observing left ventricular myocardium and endocardium approach to one-third of apex of heart. Results All 24 patients showed obvious echocardiographic findings characterized by prominent and excessive myocardial trabeculations and deep intratrabecular recesses in the affected ventricular cavity. Three cases complicated with other congenital heart malformations, 20 cases with congestive heart failure and only 2 cases were asymptomatic. Coronary arteriography was performed in 13 cases and revealed normal findings. Two cases eventually underwent heart transplantation because of severe congestive heart failure. Gross and histological findings demonstrated prominent muscular trabeculations, with deep intratrabecular recesses into lesion heart. Serial section from the base of the ventricle toward the apex revealed gradually weaker myocardium. Noncompaction of ventricular myocardium showed a thin, compacted epicardial and an extremely thickened endocardium by fibrous tissue. Conclusions Noncompaction of ventricular myocardium has characteristic echocardiographic manifestations and specific pathologic changes, which are different from those of primary enlarged type of cardiomyopathy.