摘要目的 探讨心尖肥厚型心肌病合并左心室心尖部室壁瘤的临床及心脏磁共振(CMR)特征.方法 纳入2010年1月至2017年12月在阜外医院经CMR诊断为心尖肥厚型心肌病合并左心室心尖部室壁瘤患者25例,对其临床基线资料及CMR特征进行回顾性分析.其中,单纯型心尖肥厚型心肌病(仅累及左心室心尖部,左心室近中段未受累)14例,混合型心尖肥厚型心肌病(除左心室心尖部受累以外,左心室近中段心肌亦出现增厚)1 1例.结果 在心尖肥厚型心肌病合并左心室心尖部室壁瘤患者中,20～70岁患者占84％(21/25),男性占68％(17/25).临床表现为胸闷占68％(17/25),胸痛占56％(14/25),心悸占32％(8/25),呼吸困难占16％ (4/25),晕厥占12％(3/25).患者均出现心电图ST-T改变,其中80％(20/25)患者出现T波倒置.超声心动图诊断心尖肥厚型心肌病和左心室心尖部室壁瘤的漏诊率分别为16％(4/25)和68％(17/25).CMR显示心尖肥厚型心肌病合并左心室心尖部室壁瘤患者的心尖部室壁变薄、膨出,心尖部运动消失、减弱或出现矛盾运动.76％(19/25)的患者出现瘤壁透壁性强化,其室壁瘤最大横径大于无瘤壁透壁性强化患者[(22.0±10.8)mm比(11.7±4.0)mm,P=0.033].结论 心尖肥厚型心肌病合并左心室心尖部室壁瘤有一定的临床特征,CMR可以准确、客观地评估此类疾病.

abstractsObjective To evaluate the clinical and cardiac magnetic resonance (CMR) features of apical hypertrophic cardiomyopathy (ApHCM) patients complicating with left ventricular apical aneurysm (LVAA).Methods CMR confirmed 25 ApHCM patients complicating with LVAA from January 2010 to December 2017 in Fuwai hospital were included in this study,and the baseline clinical data and CMR characteristics were retrospectively analyzed.There were 14 pure ApHCM (hypertrophy limited at the apical segments) complicating with LVAA patients and 11 mixed ApHCM (predominantly apical hypertrophy along with thickening of contiguous non-apical left ventricular region) with LVAA patients.Results In this patient cohort,age of 84％ (21/25) patients ranged between 20-70 years old,and 68％ (17/25) were male.There were 68％ (17/25) patients with complaint of chest distress symptom,56％ (14/25) with complaint of chest pain,32％ (8/25) with complaint of palpitation,16％ (4/25) with complaint of dyspnea,and 12％ (3/25) presented as syncope.ST-T segment changes of electrocardiogram were observed in all patients,and giant negative T waves were detected in 80％ patients (20/25).The rate of missed diagnosis by echocardiography for detecting ApHCM and LVAA was 16％ (4/25) and 68％ (17/25),respectively.CMR showed discrete thin-walled dyskinetic or akinetic segment of the most distal portion of the left ventricular chamber in ApHCM patients with LVAA.Transmural late gadolinium enhancement of the aneurysmal rim was detected in 76％ (19/25) patients,and the maximum transverse dimension of aneurysm was bigger in patients with transmural late gadolinium enhancement than in patients without transmural late gadolinium enhancement ((22.0± 10.8)mm vs.(11.7 ±4.0) mm,P=0.033).Conclusion ApHCM with LVAA patients have distinct cardiac clinical features,and CMR is the most useful tool for the accurate and objective evaluation of this disease.