肥厚性心肌病合并室中梗阻及顶动脉瘤1例。

K. Harada, T. Shimizu, Y. Sugishita, A. Yao, J. Suzuki, K. Takenaka, Y. Hirata, R. Nagai, T. Takahashi
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引用次数: 34

摘要

一位71岁的女性患有肥厚性心肌病,并伴有室中梗阻和左心室顶端动脉瘤。30多年来,她的心电图异常,在过去的一年里,她不时出现头晕和低血压的症状。24小时动态心电图监测显示室性阵发性收缩(676次/天)伴非持续性室性心动过速。多普勒超声心动图显示,在舒张早期,从顶端动脉瘤到左心室流出的矛盾射流。磁共振成像显示中心室肥大和不运动的薄心尖壁,血管造影证实了这一点。冠状动脉造影未见主要外冠状动脉狭窄,但有异常冠状动脉压迫,明显供应左室壁肥厚部分。应力铊-201心肌显像显示左心室尖部有持续严重的缺损。一项血流动力学研究显示心输出量低,左心室尖顶高压室和主动脉下低压室之间存在心室内压力梯度(约90mmhg)。本病例是一位罕见的老年妇女合并肥厚性心肌病、中室性梗阻和顶动脉瘤的病例。心肌缺血可能在顶端动脉瘤的发生中起重要作用。
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Hypertrophic cardiomyopathy with midventricular obstruction and apical aneurysm: a case report.
A 71-year-old woman had hypertrophic cardiomyopathy associated with midventricular obstruction and an apical aneurysm in the left ventricle. She had had abnormal electrocardiograms for more than 30 years and for the past year had been suffering from occasional attacks of dizziness and low systemic blood pressure. Holter 24-h electrocardiographic monitoring revealed ventricular paroxysmal contractions (676/day) with nonsustained ventricular tachycardia. Doppler echocardiography revealed paradoxical jet flow from the apical aneurysm to the left ventricular outflow during early diastole. Magnetic resonance imaging depicted midventricular hypertrophy and a dyskinetic thin apical wall, which were confirmed by angiography. Coronary angiograms showed no narrowing of the major extramural coronary arteries, but there was compression of aberrant coronary arteries apparently feeding the hypertrophic portion of the left ventricular wall. Stress thallium-201 myocardial imaging showed a persistent severe defect in the left ventricular apex. A hemodynamic study revealed low cardiac output and an intraventricular pressure gradient (approximately 90 mmHg) between the left ventricular apical high-pressure chamber and the subaortic low-pressure chamber. The present case represents a rare combination of hypertrophic cardiomyopathy, midventricular obstruction, and an apical aneurysm in an elderly woman. Myocardial ischemia may have played an important role in the genesis of the apical aneurysm.
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