Regional myocardial deformation in children with hypertrophic cardiomyopathy: morphological and clinical correlations
Regional myocardial deformation in children with hypertrophic cardiomyopathy: morphological and clinical correlations
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DOI：
10.1093/eurheartj/ehm444
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发表时间：
2007-12-01
影响因子：
39.3
通讯作者：
Pignatelli, Ricardo H.
中科院分区：
文献类型：
--
作者：
Ganame, Javier;Mertens, Luc;Pignatelli, Ricardo H.
肥厚型心肌病（HCM）是一种室壁厚度存在明显区域性差异的疾病.然而，心肌功能与室壁厚度之间的关系尚未得到很好的研究。超声应变率（SR）成像使研究局部心肌变形成为可能。我们调查是否区域收缩变形减少HCM的儿科患者，并评估其与壁厚，心电图模式，和exercise capability.Methods和结果的关系，我们研究了41名儿童与不对称HCM（平均年龄12.3岁）和29名对照。进行心电图、运动试验（可行时）和组织多普勒成像超声心动图。从基底隔、中隔和基底外侧心肌节段的纵向方向以及从基底前隔和下外侧心肌节段的径向方向计算收缩期峰值SR、应变、收缩后缩短和至最大应变的时间。与对照组相比，HCM儿童的所有心肌节段变形均显著减少。在肥厚型心肌病组中，与中间隔和基底外侧心肌节段相比，基底间隔的收缩期峰值SR和应变显著较低。在基底隔，收缩后缩短明显高于中间隔和侧心肌段，最大应变的时间显着延长。收缩期峰值应变与室壁厚度呈强的反曲线关系（r =-0.86，P <0.001），一旦最大室壁厚度超过3.5分，局部心肌功能没有进一步下降。室间隔基底部收缩期峰值应变与运动能力呈负相关（r = 0.68，P <0.01）。这种心肌功能的降低与最大室壁厚度和运动能力降低有关。
Aims Hypertrophic cardiomyopathy (HCM) is a disease with marked regional differences in wall thickness. However, the relation between myocardial function and wall thickness has not been well studied. Ultrasonic strain rate (SR) imaging makes it possible to study the regional myocardial deformation. We investigated whether regional systolic deformation is reduced in paediatric patients with HCM and evaluated its relation with wall thickness, electrocardiographic pattern, and exercise capacity.Methods and results We studied 41 children with asymmetric HCM (mean age 12.3 years) and 29 controls. Electrocardiograms, exercise testing (when feasible), and echocardiograms with tissue Doppler imaging were performed. Peak systolic SR, strain, post-systolic shortening, and time to maximal strain were calculated in the longitudinal direction from the basal septum, mid-septum, and basal lateral myocardial segments and in the radial direction from the basal antero-septal and infero-lateral myocardial segments. Children with HCM had a significant reduction in deformation in all myocardial segments when compared with controls. In the HCM group, peak systolic SR and strain were significantly lower in the basal septum when compared with the mid-septal and basal lateral myocardial segments. In the basal septum, post-systolic shortening was significantly higher and time to maximal strain significantly longer than in mid-septal and lateral myocardial segments. A strong inverse curvilinear relation between peak systolic strain and wall thickness was found (r = -0.86, P < 0.001), with no further decrease in the regional myocardial function demonstrated once maximal wall thickness exceeded a Z-score of 3.5. Peak systolic strain in the basal part of the septum correlated inversely with exercise capacity (r = 0.68, P < 0.01).Conclusion Systolic deformation is significantly and inhomogeneously reduced in children with HCM. This reduction in myocardial function is related to maximal wall thickness and decreased exercise capacity.