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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 characteristics of cardiac magnetic resonance imaging (CMR) of left ventricular apical hypoplasia (LVAH). Methods Seven patients diagnosed with LVAH were enrolled. All patients underwent CMR examination. The morphology of the heart was observed with black blood sequence. The T1W, T2W and T1W fat suppression sequences were used to evaluate the myocardial infiltration, myocardial motion and function of the film sequence, and the contrast enhancement delay sequence to evaluate myocardial fibrosis. All 7 patients were followed up. Results The left ventricle of 7 patients with LVAH was spherical. The right ventricle was wrapped around the left ventricle to the left, showing “banana” shape. The left ventricular papillary muscle was attached to the apical region of the left ventricle. The ventricular septum protruded to the right ventricle, Visible limitations of apex decreased or diffuse exercise weakened, ejection fraction (44.10 ± 2.86)%; apex of the normal myocardium were replaced by adipose tissue. Delayed enhanced scan showed a case of patients with interventricular septal midline visible enhancement. Of the 7 patients, 4 patients were successfully followed up for 1 to 43 months. One patient was hospitalized for heart failure, and the other 3 patients had a good prognosis. Conclusion CMR can obtain LVAH morphology, motor function and cardiac perfusion and other aspects of information, can be used for the diagnosis and differential diagnosis of LAVH.