Department of Cardiology 5F 003, Institute for Cardiovascular Research (ICaR-VU), VU University Medical Center, De Boelelaan 1117, 1081 HV, Amsterdam, The Netherlands,
Int J Cardiovasc Imaging. 2014 Mar;30(3):523-33. doi: 10.1007/s10554-014-0364-5. Epub 2014 Jan 30.
The aim is to detect early changes in myocardial mechanics in hypertrophic cardiomyopathy (HCM) mutation carriers, three-dimensional speckle tracking echocardiography (3DSTE) was used for screening of family members in the HCM population. Eighty subjects were divided as: HCM mutation carriers (n = 23), manifest HCM patients (n = 28), and normal controls (n = 29). They prospectively underwent 3DSTE for left atrial (LA) and left ventricle (LV) strain analysis. HCM mutation carriers showed significantly higher LA minimum volume (ml/m(2)) (17 ± 6 vs. 14 ± 4, respectively, P = 0.03) and a significantly lower peak atrial longitudinal strain (PALS) (%), (27 ± 5 vs. 31 ± 7, respectively, P = 0.02) when compared to controls. However, no differences were found in global or regional LV systolic myocardial deformation between both groups. Manifest HCM patients, compared to carriers showed significantly higher LA minimum (27 ± 10 vs. 17 ± 6, respectively, P < 0.001) and maximum volume (42 ± 14 vs. 32 ± 8, respectively, P = 0.007) as well as lower LA ejection fraction (%) (35 ± 8 vs. 47 ± 9, respectively, P < 0.001) and PALS (17 ± 5 vs. 27 ± 5, respectively, P < 0.001). Comparing LV strain, HCM patients showed reduced global longitudinal (-11 ± 4 vs. -16 ± 3, respectively, P < 0.001) and area strain (-35 ± 6 vs. -40 ± 5, respectively, P = 0.005). HCM mutation carriers may be distinguished from healthy subjects using 3DSTE through detection of LA dysfunction that may indicate LV diastolic dysfunction. Further research in a larger study population with gene-specific analysis is warranted for potential clinical usefulness of 3DSTE in family screening for HCM.
目的是检测肥厚型心肌病(HCM)突变携带者心肌力学的早期变化,使用三维斑点追踪超声心动图(3DSTE)对 HCM 人群中的家族成员进行筛查。80 名受试者分为:HCM 突变携带者(n=23)、有症状的 HCM 患者(n=28)和正常对照组(n=29)。他们前瞻性地接受了 3DSTE 左心房(LA)和左心室(LV)应变分析。HCM 突变携带者的 LA 最小容积(ml/m²)明显较高(分别为 17±6 和 14±4,P=0.03),峰值心房纵向应变(PALS)%明显较低(分别为 27±5 和 31±7,P=0.02)与对照组相比。然而,两组之间的整体或局部 LV 收缩期心肌变形无差异。与携带者相比,有症状的 HCM 患者的 LA 最小容积(分别为 27±10 和 17±6,P<0.001)和最大容积(分别为 42±14 和 32±8,P=0.007)明显较高,LA 射血分数(%)(分别为 35±8 和 47±9,P<0.001)和 PALS(分别为 17±5 和 27±5,P<0.001)明显较低。比较 LV 应变,HCM 患者的整体纵向应变(-11±4 和-16±3,P<0.001)和面积应变(-35±6 和-40±5,P=0.005)降低。HCM 突变携带者可能通过检测 LA 功能障碍,从而指示 LV 舒张功能障碍,与健康受试者通过 3DSTE 区分。在更大的研究人群中进行基因特异性分析,以评估 3DSTE 在 HCM 家族筛查中的潜在临床用途是必要的。