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目的 通过对四例孤立性心室肌致密化不全 (INVM)临床资料的分析以提高对此病的认识。方法 对 4例患儿的临床、心电图、胸部X线、超声心动图表现及 1例心室造影结果进行分析和总结。结果 以阵发性胸闷、气短及头晕为主要症状 ;4例均出现心脏扩大 ,心音低钝 ,2例心律不齐 ,2例心尖部Ⅱ级收缩期杂音 ;心电图均表现为心肌缺血 ,2例室性早搏 ,1例阵发性室性心动过速合并完全性右束支传导阻滞 ;胸部X线检查提示心脏扩大 ;二维超声心动图在心室内探及大量突出的肌小梁及深陷的小梁隐窝 ;彩色多普勒提示小梁间有血流与心腔相通 ,2例出现左心收缩功能下降 ,1例左心舒张功能下降 ,1例心室造影提示心腔内肌小梁增多 ,排列紊乱 ,放射状充盈缺损。结论 INVM是一种病因不明的心室肌结构异常疾病。它对心脏带来三种危险 :(1)心功能不全 ;(2 )室性心律失常 ;(3)心内膜血栓伴各系统栓塞。超声心动图是诊断INVM的一线工具。
Objective To improve the understanding of the disease through the analysis of four cases of isolated ventricular myocardial degeneration (INVM). Methods Four cases of children with clinical, electrocardiogram, chest X-ray, echocardiography and 1 case of ventricular angiography results were analyzed and summarized. The results of paroxysmal chest tightness, shortness of breath and dizziness as the main symptoms; 4 cases were enlarged heart, heart sound blunt, 2 cases of arrhythmia, 2 cases of apical systolic murmur; ECG showed myocardial ischemia, 2 Cases of premature ventricular contractions, 1 case of paroxysmal supraventricular tachycardia with complete right bundle branch block; chest X-ray examination prompted the heart to expand; two-dimensional echocardiography in the ventricle exploration and a large number of prominent trabecular and deep Trabecular cavity, color Doppler showed that blood flow between the trabeculae and the heart chamber, 2 cases of decreased left ventricular systolic function, left ventricular diastolic function decreased in 1 case, 1 case of ventricular angiography showed increased intracardiac muscle trabeculae , Disordered, radial filling defects. Conclusion INVM is an unknown disease of ventricular structural abnormalities. It carries three risks to the heart: (1) cardiac insufficiency; (2) ventricular arrhythmias; and (3) endocardial thrombosis with systemic embolism. Echocardiography is a first-line tool for diagnosing INVM.