Yang K, Zhao S H, Lu M J, Song Y Y, Li L, Chen X Y, Yin G, Wei M D
Department of Magnetic Resonance Imaging, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100037, China.
Department of Radiology, Plastic Surgery Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100144, China.
Zhonghua Xin Xue Guan Bing Za Zhi. 2019 Jul 24;47(7):534-538. doi: 10.3760/cma.j.issn.0253-3758.2019.07.005.
To evaluate the clinical and cardiac magnetic resonance (CMR) features of apical hypertrophic cardiomyopathy (ApHCM) patients complicating with left ventricular apical aneurysm (LVAA). 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. 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, 0.033). 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.
评估合并左心室心尖部室壁瘤（LVAA）的肥厚型心肌病（ApHCM）患者的临床及心脏磁共振成像（CMR）特征。本研究纳入了2010年1月至2017年12月期间在阜外医院经CMR确诊合并LVAA的25例ApHCM患者，并对其基线临床资料及CMR特征进行回顾性分析。其中，14例为单纯ApHCM（肥厚局限于心尖段）合并LVAA患者，11例为混合型ApHCM（以心尖部肥厚为主并伴有相邻非心尖左心室区域增厚）合并LVAA患者。在该患者队列中，84%（21/25）患者年龄在20至70岁之间，68%（17/25）为男性。68%（17/25）患者有胸闷症状，56%（14/25）有胸痛，32%（8/25）有心悸，16%（4/25）有呼吸困难，12%（3/25）表现为晕厥。所有患者均观察到心电图ST-T段改变，80%（20/25）患者检测到巨大倒置T波。超声心动图检测ApHCM和LVAA的漏诊率分别为16%（4/25）和68%（17/25）。CMR显示合并LVAA的ApHCM患者左心室最远端节段有离散的薄壁运动障碍或无运动节段。76%（19/25）患者检测到瘤壁透壁性延迟钆增强，有透壁性延迟钆增强患者的瘤体最大横径大于无透壁性延迟钆增强患者（（22.0±10.8）mm对（11.7±4.0）mm，P = 0.033）。合并LVAA的ApHCM患者有独特的心脏临床特征，CMR是准确、客观评估该疾病最有用的工具。
