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目的探讨123例小儿心肌致密化不全(NVM)的临床特点。方法 2005年4月至2009年9月广东省心血管病研究所收治NVM患儿123例,对所有患儿临床表现、心电图、X线胸片、超声心动图和其中31例心导管检查、24例心脏CT检查的结果进行分析和总结。结果心前区不适、气促、多汗50例,心脏扩大110例,心音低钝30例;合并其他心脏畸形65例;心电图ST-T改变104例,心律失常29例;心胸比例大于0.5者98例;超声心动图均见受累心肌肌小梁过度隆突,形成隐窝,成网状结构;行心脏CT或心导管检查的患儿均有相应的特征性影像学改变。结论小儿NVM临床主要表现为心功能不全和心律失常,各系统栓塞罕见;易合并其他心脏畸形;超声心动图是诊断NVM的首选方法 。
Objective To investigate the clinical features of 123 cases of pediatric myocardial degeneration (NVM). Methods From April 2005 to September 2009, 123 patients with NVM were enrolled in the Guangdong Provincial Institute of Cardiovascular Diseases. All children underwent clinical examination, electrocardiogram, X-ray, echocardiography and cardiac catheterization in 31 cases. 24 The results of cardiac CT examination were analyzed and summarized. The results of precordial discomfort, shortness of breath, hyperhidrosis in 50 cases, heart enlargement in 110 cases, 30 cases of heart sound low blunt; combined with other cardiac malformations in 65 cases; electrocardiogram ST-T changes in 104 cases, arrhythmia in 29 cases; 98 cases; echocardiography were involved in myocardial muscle trabecular excessive overgrowth, the formation of crypts, into a network structure; line of cardiac CT or cardiac catheterization in children with the corresponding characteristic image changes. Conclusion The main clinical manifestations of NVM in children are cardiac insufficiency and arrhythmia. The embolization of each system is rare. It is easy to combine with other cardiac malformations. Echocardiography is the first choice for the diagnosis of NVM.