Butz Thomas, Horstkotte Dieter, Langer Christoph, Esdorn Hermann, Kleikamp Georg, Körfer Reiner, Faber Lothar
Department of Cardiology, Heart Center North Rhine-Westphalia, Ruhr University Bochum, Georgstrasse 11, D-32545 Bad Oeynhausen, Germany.
Eur J Echocardiogr. 2008 Mar;9(2):344-5. doi: 10.1093/ejechocard/jen018.
Echocardiography demonstrated pronounced asymmetric left ventricular (LV) hypertrophy and thickened right ventricular muscular components in a 54-year-old woman with a history of dyspnoea (NYHA III), and recurrent syncopes. Left ventricular outflow peak gradient was 80 mmHg at rest and 125 mmHg during Valsalva manoeuvre. Cardiac cine and gadolinium-enhanced T1 weighted magnetic resonance imaging (MRI) provided striking images of a right ventricular outflow tract obstruction and a markedly abnormal gadolinium uptake in the interventricular septum consistent with myocardial fibrosis. Right and left heart catherization, with simultaneous measurement of aortic and LV pressures revealed a 150 mmHg subaortic gradient and a 130 mmHg subpulmonic gradient at rest. Impediment to right ventricular (RV) outflow was due to massive hypertrophy of the crista supraventricularis with an 'hour-glass' deformity. A surgical intervention with LV septal myotomy-myectomy and RV ventriculotomy was performed successfully. Hypertrophic obstructive cardiomyopathy with significant RV and LV outflow tract obstruction is a very rare finding. Echocardiography and MRI can be used in combination for non-invasive evaluation of morphological and haemodynamic information because mechanisms of obstruction are different in each ventricle.
超声心动图显示,一名有呼吸困难病史(纽约心脏协会心功能分级III级)且反复晕厥的54岁女性存在明显的不对称性左心室肥厚以及右心室肌成分增厚。左心室流出道静息时峰值梯度为80 mmHg,瓦尔萨尔瓦动作时为125 mmHg。心脏电影成像和钆增强T1加权磁共振成像(MRI)显示了右心室流出道梗阻的显著图像,以及室间隔明显异常的钆摄取,符合心肌纤维化表现。右心和左心导管检查同时测量主动脉和左心室压力,结果显示静息时主动脉下梯度为150 mmHg,肺动脉下梯度为130 mmHg。右心室流出道受阻是由于室上嵴巨大肥厚并呈“沙漏”样畸形。成功实施了左心室间隔切开 - 心肌切除术和右心室切开术。肥厚性梗阻性心肌病合并显著的右心室和左心室流出道梗阻是一种非常罕见的情况。超声心动图和MRI可联合用于无创评估形态学和血流动力学信息,因为每个心室的梗阻机制不同。