Veselka J
Kardiologická klinika 2, LF UK a FNM, Praha.
Cas Lek Cesk. 2006;145(4):279-83.
Hypertrophic cardiomyopathy (HCM) is a complex and relatively common genetic heart disease. HCM is caused by mutations of genes encoding sarcomeric contractile proteins and it is characterized by heterogeneous pattern of left ventricular hypertrophy with dynamic obstruction of left ventricular outflow tract. HCM is associated with both impaired left ventricular contractility and diastolic function. Using Doppler echocardiography, we are able to assess left ventricular diastolic function and measure left ventricular outflow gradient. Tissue Doppler imaging of mitral annulus is able to discriminate genotype-positive patients which allows us to improve diagnostic sensitivity of echocardiography. The Tei index is a new Doppler index, combining systolic and diastolic time intervals as an expression of global myocardial performance ("index of myocardial performance"). Non-pharmacologic treatment of obstructive HCM (alcohol septal ablation) is associated with improvement of Tei index. Doppler echocardiography is an indispensable tool in the management of HCM.
肥厚型心肌病（HCM）是一种复杂且相对常见的遗传性心脏病。HCM由编码肌节收缩蛋白的基因突变引起，其特征是左心室肥厚模式不均一，并伴有左心室流出道动态梗阻。HCM与左心室收缩功能和舒张功能受损均有关。使用多普勒超声心动图，我们能够评估左心室舒张功能并测量左心室流出梯度。二尖瓣环组织多普勒成像能够鉴别基因型阳性患者，这使我们能够提高超声心动图的诊断敏感性。Tei指数是一种新的多普勒指数，它结合了收缩期和舒张期时间间隔，作为整体心肌功能的一种表达（“心肌功能指数”）。梗阻性HCM的非药物治疗（酒精室间隔消融术）与Tei指数的改善有关。多普勒超声心动图是HCM管理中不可或缺的工具。
