实时三维超声诊断孤立性左心室心肌致密化不全一家系并文献复习

A pedigree of isolated noncompaction of left ventricular myocardium by real-time three-dimension ultrsound and literature review

目的探讨心肌致密化不全(NVM)家系分析及实时三维超声诊断该病的临床意义。方法 NVM患者家系共9人入选。应用实时三维超声观察非致密化心肌的特点、分布范围。结果该家系中NVM患者3人,均有典型的三维超声表现和不同程度的心功能减退,其中仅1人具有心衰症状,余2人无异常症状和体征;另肥厚型心肌病患者1人。结论实时三维超声是诊断NVM的较好手段,家系分析对防治NVM具有重要临床意义。

Objective To evaluate the value of pedigree analysis and real-time three-dimension ultrasound（RT3DU） in noncompaction of ventricular myocardium（NVM）.Methods Nine people in a NVM pedigree were studied.The feature and extent of noncompaction myocardium were observed by RT3DU.Results There were 3 NVM patients who had typical RT3DU appearance in this pedigree and only 1 patient had symptom of cardiac failure.In addition,there was a hypertrophic cardiomyopathy patient in this pedigree.Conclusion RT3DU is a pretty good technology for diagnosing NVM and pedigree analysis is very important in prevention and cure of NVM.