T1 Measurements Identify Extracellular Volume Expansion in Hypertrophic Cardiomyopathy Sarcomere Mutation Carriers With and Without Left Ventricular Hypertrophy
T1 Measurements Identify Extracellular Volume Expansion in Hypertrophic Cardiomyopathy Sarcomere Mutation Carriers With and Without Left Ventricular Hypertrophy
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DOI：
10.1161/circimaging.112.000333
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发表时间：
2013-05-01
影响因子：
7.5
通讯作者：
Kwong, Raymond Y.
中科院分区：
文献类型：
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作者：
Ho, Carolyn Y.;Abbasi, Siddique A.;Kwong, Raymond Y.
背景-心肌纤维化是肥厚型心肌病(HCM)的一个特征，也是心律失常和心力衰竭的潜在底物。肌节突变似乎在左心室肥厚(LVH)发生之前就会导致纤维化改变。为了进一步评估这些过程，我们使用心脏磁共振和T1测量来量化心肌细胞外体积(ECV)。方法和结果-肌节突变携带者伴LVH(G+/LVH+，n=37)和不伴LVH(G+/LVH-，n=29)，无突变的HCM患者(肌节阴性HCM，n=11)和健康对照组(n=11)接受对比心脏磁共振检查，测量注射Gd前后的T1时间。同时，超声心动图和血清胶原合成、血流动力学应激和心肌损伤的生物标志物也在一个子集中可用。与对照组比较，G+/LVH+、G+/LVH-、G+/LVH突变携带者ECV分别为0.36+/-0.01、0.33+/-0.01、0.27+/-0.01，差异有统计学意义(P
Background-Myocardial fibrosis is a hallmark of hypertrophic cardiomyopathy (HCM) and a potential substrate for arrhythmias and heart failure. Sarcomere mutations seem to induce profibrotic changes before left ventricular hypertrophy (LVH) develops. To further evaluate these processes, we used cardiac magnetic resonance with T1 measurements on a genotyped HCM population to quantify myocardial extracellular volume (ECV).Methods and Results-Sarcomere mutation carriers with LVH (G+/LVH+, n=37) and without LVH (G+/LVH-, n=29), patients with HCM without mutations (sarcomere-negative HCM, n=11), and healthy controls (n=11) underwent contrast cardiac magnetic resonance, measuring T1 times pre- and postgadolinium infusion. Concurrent echocardiography and serum biomarkers of collagen synthesis, hemodynamic stress, and myocardial injury were also available in a subset. Compared with controls, ECV was increased in patients with overt HCM, as well as G+/LVH-mutation carriers (ECV=0.36 +/- 0.01, 0.33 +/- 0.01, 0.27 +/- 0.01 in G+/LVH+, G+/LVH-, controls, respectively; P