孤立性左心室心肌致密化不全磁共振图像的诊断特征

Magnetic resonance imaging for diagnosis of isolated left ventricular noncompaction

目的:探讨孤立性左心室心肌致密化不全(Isolated left ventricular noncompaction,ILVNC)心脏MRI的诊断特征。方法:2014年1—12月我院心内科门诊及住院患者共计8 895例行心脏超声心动图检查。排除其他合并疾病考虑ILVNC者行心脏MRI扫描,满足心脏超声及心脏MRI双重诊断标准及临床综合考虑纳入ILVNC组患者6例,分析临床资料及心脏MRI图像特点。结果:6例患者表现进行性心功能不全,伴有左心室扩大。ILVNC的心脏MRI诊断特征:左心室心肌分为两层,非致密化心肌层肌小梁粗大且排列不整齐,舒张末期可见隐窝充盈血液;心肌非致密化受累节段多,心尖部100%受累;舒张末期非致密化心肌与致密化心肌厚度比值超过2.3。结论:基于心脏MRI能够更准确全面展示非致密化心肌层可用于ILVNC诊断与鉴别,需要进一步行多中心大样本研究。

Objective： To evaluate cardiac MRI for diagnosis of isolated left ventricular noncompaction （ILVNC）. Methods： A total of 8 895 patients underwent echocardiography in our hospital from January to December 2014. Patients suspected to have ILVNC underwent cardiac MRI. Finally, six ILVNC patients were enrolled after a general consideration of manifestations of echocardiography, cardiac MRI and clinical data. Results： The clinical presentations of 6 ILVNC patients were heart failure with left ventricular enlargement. Cardiac MRI showed： two-layered myocardial structure with compacted and thickened non- compacted layer which consisted of trabecutar meshing with deep endocardial spaces. The apex was involved in all cases. The intertrabecular recesses were perfused with blood at cardiac MRI end-diastole phase. The maximum end-diastole thickness ra- tio of noncompacted layer to compacted layer was more than 2.3. Conclusion： Cardiac MRI provides more accurate and reli- able evaluation of noncompacted myocardium for ILVNC diagnosis. Further investigation is needed in large group of patients.