孤立性左心室肌致密化不全10例分析

Isolated noncompaction of the left ventricular myocardium (a report of 10 cases)

目的 评价超声心动图技术在诊断孤立性左室肌致密化不全中的作用。方法 10例孤立性左室肌致密化不全患者 ,均为男性 ,年龄 2～ 6 0 (39± 17.7)岁。分别采用美国HP 5 5 0 0及日本Toshiba 140A型二维及多普勒超声心动图仪进行检查 ,探头频率 2 .5 3 .5MHz。部分患者接受超高速CT或磁共振成像检查。结果 10例患者均有程度不同的左心功能不全、心律失常等临床表现 ,未见栓塞征象。孤立性左室肌致密化不全超声心动图特征如下 :(1)二维超声心动图可见多发性突入心室腔内的肌小梁 ,且呈节段性分布。好发于左室心尖部、前侧壁 ;(2 )多普勒超声心动图显示深陷于肌小梁隐窝间的血流与左室腔交通 ;(3)左心室壁呈非均匀性增厚或变薄。 10例患者均符合上述诊断标准。 2例行超高速CT检查 ,2例行磁共振成像检查 ,4例患者均可见左室心尖部、前侧壁肌小梁粗大 ,左心室腔明显扩大 ;小梁区心室壁明显增厚。 10例患者均因左心功能不全给予常规利尿剂、血管扩张剂及抗凝治疗。结论 孤立性左心室肌致密化不全是一种罕见的先天性心肌病 ,临床上可表现为渐进性左心功能不全、致命性心律失常及栓塞征象。超声心动图是无创诊断孤立性左室肌致密化不全的准确。

Objective To assess the diagnostic role of echocardiography in patients with isolated noncompaction of the left ventricular myocardium. Methods This study included ten patients aged 2 to 60 years. Studies were performed with the Hewlett Packard Sonos 5500 and Toshiba 140A echocardiography machines with imaging and Doppler transducers (2.5 3.0 MHz). Four of ten patients underwent fast computed tomography and magnetic resonance imaging examination. Results Clinical manifestations included gradually depressed left ventricular function,severe ventricular arrhythmia, and no systemic embolism. Echocardiography diagnostic criterias of INLVM were based on the following findings(1) multiple prominent ventricular trabeculation, (2) multiple deep intertrabeculation recesses communicating with the left ventricular cavity, as demonstrated by two dimensional and color Doppler flow imaging. These findings typically are most prominent in the apical and middle anterior lateral portions of the left vantricle. There were no coexisting intracardiac abnormalities. Conclusions Noncompaction of the left ventricular myocardium is a rare congenital cardiomyopathy characterized by an excessively prominent trabecular meshwork. It is accomanied by depressed left ventricular function, ventricular arrhythmia and systemic embolism. Echocardiography is a reliable diagnostic modality of choice for noncompaction of the left ventricular myocardium.