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目的:研究心肌致密化不全患者临床特征。方法:分析超声心动图诊断为心肌致密化不全22例患者的临床特点、心电图、X线、超声心动图。结果:22例患者中对14例先症者3代直系亲属进行心脏超声检查,共发现3个家系,首发症状者均为儿童;20例患者有不同程度的左心功能不全、心律失常等临床表现,但未发现栓塞患者;17例患者12导联心电图有异常改变,主要表现为传导阻滞、室性期前收缩、异常Q波及STT改变;超声心动图示,主要累及左室,4例患者局限左室心尖部或左室后壁或下壁,1例累及右室,引起心脏增大、心室收缩舒张功能减退及瓣膜关闭不全。结论:心肌致密化不全是一种先天性心脏病,临床表现主要为心力衰竭和心律失常,超声心动图有其典型特征。
Objective: To study the clinical features of patients with myocardial densification. Methods: The clinical features, electrocardiogram, X-ray and echocardiography were analyzed in 22 cases diagnosed as myocardial densification by echocardiography. Results: Of the 22 patients, 14 cases of first-mover third-generation immediate family members were examined by echocardiography. Three families were found. The first symptom was children. Twenty patients had different degrees of left ventricular dysfunction and arrhythmia , But no embolized patients were found. The abnormal changes of 12-lead electrocardiogram were found in 17 patients, which mainly manifested as conduction block, premature ventricular contraction, abnormal Q wave and STT changes. Echocardiography mainly involved left ventricle, 4 Patients with localized apex of the left ventricle or the posterior wall or inferior wall of the left ventricle, 1 case involving the right ventricle, causing increased heart, ventricular systolic and diastolic dysfunction and valvular insufficiency. Conclusion: Myocardial densification is a congenital heart disease. The main clinical manifestations are heart failure and arrhythmia. Echocardiography has its typical features.