【摘要】 目的 比较超声心动图和磁共振成像(MRI)对心尖肥厚型心肌病(AHCM)的诊断价值，探讨AHCM的MRI影像学特征。方法 选择2016年1月至2021年5月濮阳市安阳地区医院经心电图检查疑似AHCM的78例患者为研究对象，患者均行超声心动图和MRI检查。比较超声心动图和MRI检查对AHCM诊断的灵敏度、特异度、阳性预测值和阴性预测值，以及典型心尖肥厚型心肌病(T-AHCM)组和早期心尖肥厚型心肌病(P-AHCM)组患者心脏MRI功能参数，包括心尖部室壁厚度、心尖角、左心房前后径、左心室横径、左心室舒张末容积及左心室射血分数。结果 经基因诊断或心肌组织病理标准确诊43例。超声心动图诊断AHCM真阳性26例，假阳性4例，假阴性17例，真阴性31例。MRI诊断AHCM真阳性40例，假阳性0例，假阴性3例，真阴性35例。超声心动图诊断AHCM漏诊率为39.5%(17/43),MRI诊断漏诊率为6.9%(3/43)。MRI诊断AHCM的准确度、敏感度、特异度、阳性预测值及阴性预测值显著高于超声心动图(P<0.05)。43例确诊患者心脏MRI均显示左心室心尖部及邻近节段室壁不同程度增厚，类似扑克牌“黑桃尖”样改变；P-AHCM患者左心室心尖部室壁肥厚程度相对较轻，心尖部心腔变小。P-AHCM占55.8%(24/43),T-AHCM占44.2%(19/43);单纯型AHCM占74.4%(32/43),混合型AHCM占25.6%(11/43)。超声心动图诊断AHCM漏诊的17例患者中，P-AHCM患者占30.2%(13/43),T-AHCM患者占9.3%(4/43);MRI诊断AHCM漏诊的3例患者均为P-AHCM患者，因患者心尖部室壁厚度未达诊断标准而漏诊。T-AHCM组患者心尖部室壁厚度、左心房前后径及左心室射血分数高于P-AHCM组，心尖角小于P-AHCM组(P<0.05);P-AHCM组与T-AHCM组患者左心室横径、左心室舒张末容积比较差异无统计学意义(P>0.05)。T-AHCM组和P-AHCM组首过灌注均为阴性，延迟增强扫描17例患者出现肥厚心肌内或心内膜下延迟强化；T-AHCM占63.2%(12/19),P-AHCM占20.1%(5/24);T-AHCM组增厚心肌强化阳性率高于P-AHCM组(χ~2=6.275,P<0.05)。结论 MRI对AHCM诊断价值较高，特别是P-AHCM,可作为超声心动图诊断AHCM的有效补充手段。更多还原

【Abstract】 Objective To compare the diagnostic value of echocardiogram and magnetic resonance imaging(MRI) in apical hypertrophic cardiomyopathy(AHCM),and discuss the characteristics of MRI in AHCM.Methods A total of 78 suspected of AHCM by electrocardiographic examination admitted to the Anyang District Hospital of Puyang City from January 2016 to May 2021 were selected as the research subjects.All research subjects were underwent echocardiogram and MRI.The sensitivity, specificity, positive predictive value and negative predictive value of echocardiography and MRI in AHCM were compared, and the cardiac MRI functional parameters including the apical ventricular wall thickness, apical angle, left atrial anterior posterior diameter, left ventricular transverse diameter, left ventricular end diastolic volume and left ventricular ejection fraction of the patients were compared between the pre-apical hypertrophic cardiomyopathy(P-AHCM) group and typical apical hypertrophic cardiomyopathy(T-AHCM) group.Results A total of 43 patients were diagnosed by the gene diagnosis or myocardial histopathological.Echocardiographic diagnosis of AHCM was true positive in 26 cases, false positive in 4 cases, false negative in 17 cases and true negative in 31 cases.MRI diagnosis of AHCM was true positive in 40 cases, false positive in 0 case, false negative in 3 cases and true negative in 35 cases.The missed diagnosis rate of echocardiography in the diagnosis of AHCM was 39.5%(17/43),the missed diagnosis rate of MRI in the diagnosis of AHCM was 6.9%(3/43).The accuracy rate, sensitivity, specificity, positive predictive value and negative predictive value of MRI in diagnosing AHCM were significantly higher than those of echocardiogram(P<0.05).The cardiac MRI of 43 patients diagnosed with AHCM showed left ventricular apex and adjacent segment ventricular wall thickening of different level.MRI showed “ace of spades” morphology of the left ventricle in the T-AHCM patients.MRI showed apical slightly thickened and heart cavity smaller of left ventricle in the P-AHCM patients.P-AHCM accounted for 55.8%(24/43),T-AHCM accounted for 44.2%(19/43);simplex AHCM accounted for 74.4%(32/43),hybrid AHCM accounted for 25.6%(11/43).Among the 17 patients with missed diagnosis of AHCM by echocardiography, 30.2%(13/43) were P-AHCM and 9.3%(4/43) were T-AHCM;the 3 patients missed diagnosis of AHCM by MRI were all P-AHCM,and the diagnosis was missed because the thickness of apical ventricular wall did not meet the diagnostic criteria.The apical thickness, left atrium diameter and left ventricular ejection fraction of T-AHCM patients were significantly higher than those in the P-AHCM group, the apical angle was significantly less than that in the P-AHCM group(P<0.05).There was no significant difference in the left ventricular diameter and left ventricular end-diastolic volume of patients between the P-AHCM group and T-AHCM group(P>0.05).The first ever perfusion were negative in the P-AHCM group and T-AHCM group.The hypertrophy myocardial or under the endocardium were delayed reinforcement by contrast enhancement scanning in 17 patients; among them, T-AHCM accounted for 63.2%(12/19),P-AHCM accounted for 20.1%(5/24).The positive rate of thickened myocardium enhancement of patients in the T-AHCM group was significantly higher than that in the P-AHCM group(P<0.05).Conclusion The diagnostic value of MRI for AHCM is higher than that of echocardiogram, especially for P-AHCM,and which can be used as an effective supplementary method for echocardiogram in the diagnosis of AHCM.更多还原