心肌致密化不全患者的临床特征及随访观察

The clinical features and follow-up of the patients with noncompaction of ventricular myocardium

目的观察心肌致密化不全(NVM)患者的临床特征及随访结果。方法对超声心动图诊断的38例NVM患者,进行包括症状、心电图和超声心动图的定期随访,观察其临床特征和随访结果。结果38例患者9例呈家族集聚发病,21例为散发病例,8例未进行家系筛查。资料完整者34例,左室进行性增大17例,血栓或血栓事件5例,其中死亡1例。严重的心力衰竭、心房颤动、缓慢性心律失常的患者血栓或血栓事件发生率高。结论NVM病情进展较快,血栓形成事件率高,预后差。

Objective To investigate the clinical features, prognosis and the occurrence of thromboembolism and the risk factors of thrombosis in the patients with noncompaction of ventricular myocardium （NVM）. Methods Thirty-eight patients with NVM were followed-up by examining the echocardiography, electrocardiogram, ambulatory electrocardio- graphy. The data of 34 patients was obtained. The relation was analyzed between of thromboembolism and heart function or arrhythmia. Results Familial occurrence were found in 9 patients,sporadic were found in 21 patients,pedigree analyses were not used in other 8 patients. In the follow-up, one patient died. Thrombus or thromboembolic were noted in five pa- tients, the left ventricles were enlarged in 17 patients. It was related that the occurrence of thromboembolism and atrial fibrillation,bradyarrhythmia, and low left ventricular ejection fraction. Conclusion The prognosis of NVM with heart failure,atrial fibrillation, bradyarrhythmia is poor.