心血管MRI在评价左心室心尖发育不良中的应用

Application of cardiac MRI in evaluation of left ventricular apical hypoplasia

目的 分析左心室心尖发育不良（LVAH）的心脏磁共振（CMR）成像特点。方法 收集7例诊断为LVAH的患者,所有患者均接受CMR检查。应用黑血序列观察心脏的形态结构,T1W、T2W及T1W脂肪抑制序列用于评价心肌脂肪浸润,电影序列进行心脏运动及功能的评估,对比剂延迟强化序列评价心肌纤维化。对7例患者均进行临床随访。结果 7例LVAH患者左心室呈球形,右心室向左包绕左心室心腔,呈“香蕉”状;左心室乳头肌附着于左心室心尖区域;室间隔向右心室侧凸出,并可见局限性的心尖运动减弱或弥漫性运动减弱,射血分数为（44.10±2.86）%;心尖部正常心肌均被脂肪组织代替。延迟增强扫描显示1例患者室间隔中段可见肌壁间线样强化。7例中,对4例患者成功进行随访,随访时间1-43个月,1例患者因心力衰竭入院,余3例患者预后较好。结论 CMR可获得LVAH形态学、运动功能及心脏灌注等多方面信息,可用于LAVH的诊断及鉴别诊断。

Objective To analyze the features of left ventricle apical hypoplasia （LVAH） on cardiac magnetic resonance （CMR）. Methods Seven patients diagnosed with LVAH were included. All of the patients underwent CMR examinations. Black blood sequence was used for observation of morphology, T1W, T2W and fat-saturated T1W sequence were combined to diagnose myocardial fatty infiltration, cine sequence was applied for assessment of cardiac motion and function, and con- trast agent delayed-enhancement sequence was for evaluation of myocardial fibrosis. Patients were programmed to clinical follow-up. Results LVAH indicated spherical left ventricle, with right ventricle surrounding left ventricular analogous to the shape of ＂banana＂, papillary muscles were attached to the ventricular apical zones in 7 patients. Inter-ventricular sep- turns convexed to right ventricle in 7 patients. All of them showed reduced focal apical motions or diffusely reduced wall motions of myocardium. Ejection fraction were （44.10±2.86）%. Adipose tissue substituted for normal tissue of apex in 7 patients, 1 patient showed linear enhancement in ventrieular septum. Clinical follow-up were successfully conducted in 4 of 7 patients, with the duration up to 43 months. One patient was hospitalized for heart failure, and the other 3 patients showed good prognosis. Conclusion The information on morphology, motion and function, cardiac perfusion of LVAH can be obtained with CMR, which can be applied for diagnosis and differential diagnosis of LVAH.