Cardiac magnetic resonance imaging of myocardial contrast uptake and blood flow in patients affected with idiopathic or familial dilated cardiomyopathy
Cardiac magnetic resonance imaging of myocardial contrast uptake and blood flow in patients affected with idiopathic or familial dilated cardiomyopathy
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DOI：
10.1152/ajpheart.00429.2008
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发表时间：
2008-09-01
影响因子：
4.8
通讯作者：
Hershberger, Ray E.
中科院分区：
文献类型：
--
作者：
Jerosch-Herold, Michael;Sheridan, David C.;Hershberger, Ray E.
特发性扩张型心肌病（IDC）的特征是左心室（LV）扩大伴收缩功能障碍，排除其他原因。当遗传时，它代表家族性扩张型心肌病（FDC）。我们假设IDC或FDC将显示心脏磁共振（CMR）在稳态时增加钆造影剂的心肌蓄积，并且与正常心肌相比，由于细胞外基质的结构改变而降低基线心肌血流量（MBF）。在9名IDC/FDC患者中进行了CMR。健康对照来自一般人群（n = 6）或FDC患者的未受影响的家庭成员（n = 3），无IDC/FDC的体征或症状或任何结构性心脏异常。通过T1测量确定钆对比剂的心肌分配系数（lambda（Gd））。通过标准CMR方法评估LV形状和功能以及MBF。与健康对照组相比，IDC/FDC患者的λ（Gd）升高（λ（Gd）= 0.56 +/- 0.15 vs. 0.41 +/- 0.06; P = 0.002），并与LV扩大相关（λ（Gd）与以身高为指标的舒张末期容积的相关系数为0.61; P < 0.01）和与射血分数的相关系数为-0.80; P < 0.001）。IDC患者的细胞外容积分数高于健康对照组（0.31 +/- 0.05 vs. 0.24 +/- 0.03; P = 0.002）。IDC患者的静息MBF（0.64 +/- 0.13 vs. 0.91 +/- 0.22; P = 0.01）低于未受影响的对照组，并与分配系数（r = -0.57; P = 0.012）和细胞外容积分数（r = -0.56; P = 0.019）相关。细胞外间隙的扩张与MBF减少和心室扩张相关。细胞外基质的扩张可能是IDC患者收缩功能障碍的关键因素。
Idiopathic dilated cardiomyopathy (IDC) is characterized by left ventricular (LV) enlargement with systolic dysfunction, other causes excluded. When inherited, it represents familial dilated cardiomyopathy (FDC). We hypothesized that IDC or FDC would show with cardiac magnetic resonance (CMR) increased myocardial accumulation of gadolinium contrast at steady state and decreased baseline myocardial blood flow (MBF) due to structural alterations of the extracellular matrix compared with normal myocardium. CMR was performed in nine persons affected with IDC/FDC. Healthy controls came from the general population (n = 6) or were unaffected family members of FDC patients (n = 3) without signs or symptoms of IDC/FDC or any structural cardiac abnormalities. The myocardial partition coefficient for gadolinium contrast (lambda(Gd)) was determined by T1 measurements. LV shape and function and MBF were assessed by standard CMR methods. lambda(Gd) was elevated in IDC/FDC patients vs. healthy controls (lambda(Gd) = 0.56 +/- 0.15 vs. 0.41 +/- 0.06; P = 0.002), and correlated with LV enlargement (r = 0.61 for lambda(Gd) vs. end-diastolic volume indexed by height; P < 0.01) and with ejection fraction (r = -0.80; P < 0.001). The extracellular volume fraction was higher in IDC patients than in healthy controls (0.31 +/- 0.05 vs. 0.24 +/- 0.03; P = 0.002). Resting MBF was lower in IDC patients (0.64 +/- 0.13 vs. 0.91 +/- 0.22; P = 0.01) than unaffected controls and correlated with both the partition coefficient (r = -0.57; P = 0.012) and the extracellular volume fraction (r = -0.56; P = 0.019). The expansion of the extracellular space correlated with reduced MBF and ventricular dilation. Expansion of the extracellular matrix may be a key contributor to contractile dysfunction in IDC patients.