左心室心尖发育不良的临床和影像学特征

Clinical and cardiac imaging characteristics of patients with left ventricular apical hypoplasia

目的探讨左心室（左室）心尖发育不良的临床和影像学特征。方法入选2008年1月至2012年1月诊断为左室心尖发育不良的患者7例（男性3例，女性4例），年龄6～44（19．9±14．2）岁。患者均接受经胸超声一tD动图检查；其中3例患者接受心血管MRI检查；2例患者接受CT检查，其中1例患者同时接受心导管和造影检查。分析患者的临床和影像学特征。结果7例患者中，以胸闷不适就诊4例，3例患者心前区可闻及杂音。心电图显示3例患者合并心房颤动。影像学检查显示，患者的左室心尖部均扁平，心腔呈球形改变；左室横径均增大而纵径相对短缩，横径为（57．9±11．6）mm；室间隔均凸向右室，厚度为（7．3±1．2）mm；左室射血分数为（47．6±17．2）％，其中2例患者的左室射血分数减低；左室乳头肌均附着于扁平的左室前壁心尖部；右室腔均增大并包绕发育不良的左室心尖部，右室横径为（19．7±7．6）mm；接受心血管MRI或CT检查的5例患者均在心尖部存在脂肪替代。结论左室一tD尖发育不良的临床表现各异，影像学以左室心尖部扁平、心腔球形改变、左室乳头肌异常附着于前壁心尖部以及右室远端包绕发育不良的左室心尖为特征。

Objective To analyze the clinical and cardiac imaging characteristics of patients with left ventricular apical hypoplasia （LVAH）. Methods From January 2008 to January 2012, seven patients [3 male/4 female, age： 6 - 44 （ 19.9 ± 14.2） years ] with LVAH were included in this cohort. Transthoracic echocardiogram was performed in all patients, cardiovascular MRI was performed in 3 patients and cardiovascular CT in another 2 patients. In addition, one LVAH patient underwent cardiac catheterization and angiography examination. Results Four out of 7 patients complained chest discomfort. Precordial murmur was heard in 3 patients. Atrial fibrillation was evidenced by electrocardiogram in 3 patients. Left ventricular end-diastolic diameter [ （57. 9± 11.6 ）mm ] increased while left ventricule （LV） longitudinal diameter reduced in all patients. Left ventrieular systolic function was reduced in 2 patients and mean LVEF was （47.6 ± 17.2 ）%. The interventricular septum bulged towards the right, and the ventricular septum thickness was （7.3 ± 1.2 ）mm. The papillary muscles were dominant on the flattened LV anteroapical region. The fight ventricle elongated and wrapped around the hypoplastic left ventricular apex, and the dimension of right ventricle was （ 19. 7 ± 7.6 ） mm. Focal fat replacement of the left ventricular apical wall was evidenced in 5 patients underwent cardiovascular MRI or CT examinations. Conclusions Clinical symptoms are non-specific in LVAH patients. Truncated and spherical LV, abnormal origin of papillary muscles in the flattened LV anterior apex and an elongated right ventricle wrapping around the LV apex as well as focal fat replacement of the left ventrieular apical wall are typical imaging characteristics of LVAH.