心尖肥厚型心肌病18例报告

ANALYSIS OF 18 CASES WITH APICAL HYPERTROPHIC CARDIOMYOPATHY

心尖肥厚型心肌病属于非梗阻型心肌病的一种，临床少见。本文对１８例患者的临床表现、心电图、超声心动图及磁共振成像特点进行了分析。心电图的典型改变是胸前导联巨大倒置的Ｔ波，高ＱＲＳ波群；超声心动图特点为心尖部肥厚或闭塞，左室流出道无梗阻。磁共振成像证实心尖部心肌肥厚。心室造影呈＂铲形＂（Ｓｐａｄｅ－ｌｉｋｅ）。该病常被误诊为＂冠心病＂、＂心内膜下心肌梗塞＂。国外有关报道指出，以心电图倒置Ｔ波深度＞１０ｍｍ做为诊断该病的标准。而本文作者却观察到，一部分患者心电图倒置Ｔ波深度＜１０ｍｍ，且有的患者心室造影不呈＂铲形＂。故作者认为后者是该病的一种新的类型。超声心动图是诊断该病的重要手段，必要时可做磁共振成像检查。该病对血液动力学影响较小，因影响左室舒张功能，故引起一些临床症状。该病预后一般较好。

Apical hypertrophic cardiomyopathy is a special type of hypertrophic cardiomyopathy (HCM) , which belongs to nonobstructive HCM. The clinical, electrocardiographic and echocardiographic features were analysed in 18 cases of apical HCM. The major diagnostic features of apical HCM are (1) electrocardiographic‘giant’negative T wave with high QRS voltage, (2) echocardiographic increase in thickness of ventricular wall or obliteration of ventricular cavity in the apical segment, (3) angiocardiographic‘pade-like’configuration with marked apical obliteration in the left ventricle during diastole, (4) apical hypertrophy by MRI.The patients are often suspected to have ischemic heart disease. We found that negative T wave<10 mm in the electrocardiography did not exclude apical HCM, it is different from that reported in the literature.It suggests that echocardiography is important for the detection of this disease. MRI might be performed if necessary. Apical HCM causes no remarkable obstruction of left ventricular (LV) outflow, but it might induce diastolic dysfunction of the LV.