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[心脏轻链淀粉样变性和高嗜酸性粒细胞综合征中的左心室变形。]

[Left ventricular deformation in cardiac light-chain amyloidosis and hypereosinophilic syndrome. ].

作者信息

Kormányos Árpád, Domsik Péter, Kalapos Anita, Marton Imelda, Földeák Dóra, Modok Szabolcs, Gyenes Nándor, Borbényi Zita, Nemes Attila

机构信息

Szent-Györgyi Albert Klinikai Központ, II. Belgyógyászati Klinika és Kardiológiai Központ,Szegedi Tudományegyetem, Általános Orvostudományi KarSzeged, Semmelweis u. 8., 6725.

Szent-Györgyi Albert Klinikai Központ, Transzfuziológiai Intézet,Szegedi Tudományegyetem, Általános Orvostudományi KarSzeged.

出版信息

Orv Hetil. 2020 Feb;161(5):169-176. doi: 10.1556/650.2020.31584.

Abstract

Hypereosinophilic syndrome (HES) and immunoglobulin light-chain amyloidosis (ALA) are two, rare haematological disorders associated with cardiac alterations. The goal of the present study was a comparative assessment of left ventricular (LV) deformational parameters in HES and ALA patients using three-dimensional speckle-tracking echocardiography (3DSTE). In the present study, results of 10 HES patients (mean age: 60.9 ± 14.7 years) and 19 ALA patients (mean age: 63.4 ± 7.8 years, 13 males) were analysed. The control group contained 13 age- and gender-matched healthy adults (mean age: 59.2 ± 4.3 years, 5 males). All patients underwent a complete two-dimensional Doppler echocardiography followed by 3DSTE. All basal segmental LV strains were significantly reduced in ALA patients as compared to the control group. Global and mean segmental LV longitudinal strain (LS) values of ALA patients proved to be significantly decreased as compared to those of the healthy control group. During comparison of HES patients and healthy controls, significant difference could be detected in global LV-LS, while segmental basal LV-LS was also significantly reduced in HES patients. Basal LV radial and 3D strains showed significant differences when parameters of HES and ALA patient groups were compared. 3DSTE is a feasible tool for the detailed assessment of LV deformation in HES and ALA patients. Significant LV deformational abnormalities could be detected in both groups. In the case of ALA, these abnormalities are more prominent. Orv Hetil. 2020; 161(5): 169-176.

摘要

高嗜酸性粒细胞综合征(HES)和免疫球蛋白轻链淀粉样变性(ALA)是两种与心脏改变相关的罕见血液系统疾病。本研究的目的是使用三维斑点追踪超声心动图(3DSTE)对HES和ALA患者的左心室(LV)变形参数进行比较评估。在本研究中,分析了10例HES患者(平均年龄:60.9±14.7岁)和19例ALA患者(平均年龄:63.4±7.8岁,13例男性)的结果。对照组包括13名年龄和性别匹配的健康成年人(平均年龄:59.2±4.3岁,5例男性)。所有患者均接受了完整的二维多普勒超声心动图检查,随后进行了3DSTE检查。与对照组相比,ALA患者所有基底节段的左心室应变均显著降低。与健康对照组相比,ALA患者的整体和平均节段左心室纵向应变(LS)值显著降低。在比较HES患者和健康对照组时,在整体左心室LS中可检测到显著差异,而HES患者的基底节段左心室LS也显著降低。比较HES和ALA患者组的参数时,基底左心室径向应变和三维应变显示出显著差异。3DSTE是详细评估HES和ALA患者左心室变形的可行工具。两组均能检测到显著的左心室变形异常。在ALA患者中,这些异常更为突出。《匈牙利医学周报》。2020年;161(5): 169 - 176。

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