Left Ventricular Structure and Function in Transthyretin- Related Versus Light- Chain Cardiac Amyloidosis
Left Ventricular Structure and Function in Transthyretin- Related Versus Light- Chain Cardiac Amyloidosis
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DOI：
10.1161/circulationaha.113.006242
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发表时间：
2014-05-06
期刊：
影响因子：
37.8
通讯作者：
Falk, Rodney H.
中科院分区：
文献类型：
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作者：
Quarta, Candida Cristina;Solomon, Scott D.;Falk, Rodney H.
免疫球蛋白淀粉样蛋白轻链（AL）相关的心脏淀粉样变性（CA）的预后比野生型（ATTRwt）或突变型（ATTRm）转甲状腺素（TTR） CA更差。详细的超声心动图研究已在AL淀粉样变性中进行，但在TTR淀粉样变性中尚未进行，这可能有助于了解这种差异。我们评估了大量CA患者的心脏结构、功能和预后，并比较了TTR和al相关疾病的结果。方法和结果我们通过标准超声心动图和二维斑点跟踪成像衍生的左心室（LV）纵向（LS）、径向（LS）和周向应变分析了172例CA （AL淀粉样变性，n=80; ATTRm, n=36; ATTRwt, n=56）。尽管保留了左室射血分数（5512%），但CA患者的左室功能严重受损。随着壁厚的增加，左室功能的标准测量和斑点跟踪成像恶化，而无论CA的发病机制和壁增厚程度如何，顶端LS都得以保留。与ATTRm和AL淀粉样变相比，attrt的特点是左室壁厚更大，射血分数更低。LS在attrt和AL淀粉样变中均比ATTRm （-15 +/- 4%， P=0.54）更低（分别为-11 +/- 3%和-12 +/- 4%，P=0.54）
Background Immunoglobulin amyloid light-chain (AL)-related cardiac amyloidosis (CA) has a worse prognosis than either wild-type (ATTRwt) or mutant (ATTRm) transthyretin (TTR) CA. Detailed echocardiographic studies have been performed in AL amyloidosis but not in TTR amyloidosis and might give insight into this difference. We assessed cardiac structure and function and outcome in a large population of patients with CA and compared findings in TTR and AL-related disease.Methods and Results We analyzed 172 patients with CA (AL amyloidosis, n=80; ATTRm, n=36; ATTRwt, n=56) by standard echocardiography and 2-dimensional speckle-tracking imaging-derived left ventricular (LV) longitudinal (LS), radial, and circumferential strains. Despite a preserved LV ejection fraction (5512%), LS was severely impaired in CA. Standard measures of LV function and speckle-tracking imaging worsened as wall thickness increased, whereas apical LS was preserved regardless of the pathogenesis of CA and the degree of wall thickening. Compared with ATTRm and AL amyloidosis, ATTRwt was characterized by greater LV wall thickness and lower ejection fraction. LS was more depressed in both ATTRwt and AL amyloidosis (-11 +/- 3% and -12 +/- 4%, respectively, P=0.54) than in ATTRm (-15 +/- 4%, P