Evaluation of global and regional myocardial work by echocardiography in patients with Fabry disease.

IF 3.4 2区 医学 Q2 GENETICS & HEREDITY
Han Wang, Ying Yang, Lin Liu, Yawen Zhao, Yang Li, Wei Zhang, Wei Ma
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Abstract

Background: This study aimed to quantitatively evaluate the left ventricular global and regional myocardial work of patients in Fabry disease (FD) by echocardiographic pressure-strain loop (PSL) analysis.

Results: The study included 48 patients with FD and 48 healthy controls matched for age and sex. According to the presence/absence of left ventricular hypertrophy (LVH), the patients with FD were divided into an LVH + group and an LVH- group. Left ventricular blood pressure was estimated noninvasively according to echocardiographic valvular events and systolic pressure in the brachial artery. Left ventricular myocardial work parameters were acquired by echocardiographic pressure-strain loop analysis. The FD groups had a significantly lower global longitudinal strain (GLS), global work index, global work efficiency (GWE), global constructive work and higher global waste work than the control group (P < .05). Regional analysis showed that all segmental myocardial waste work increased and myocardial work efficiency decreased in the LVH + group than in the LVH- group (P < .05). Segmental longitudinal strain, myocardial work index, and myocardial constructive work were markedly lower in the basal and middle segments (P < .05) and preserved in the apical segments. Multivariate analysis revealed that GWE and GLS were significant related to LVH.

Conclusions: Myocardial work analysis can be used to assess global and regional myocardial work in patients with FD. In this study, GLS and GWE were reduced in patients with FD and associated with the presence of LVH. Basal and middle myocardial work decreased in relation to the LVH, while apical myocardial work remained, which added value to explore the distribution of myocardial impairment.

通过超声心动图评估法布里病患者的整体和区域心肌功。
背景:本研究旨在通过超声心动图压力应变环(PSL)分析,定量评估法布里病(FD)患者左心室整体和区域心肌功:本研究旨在通过超声心动图压力应变环(PSL)分析,定量评估法布里病(FD)患者左心室整体和区域心肌功:研究包括 48 名法布里病患者和 48 名年龄和性别匹配的健康对照者。根据是否存在左心室肥厚(LVH),FD 患者被分为 LVH + 组和 LVH- 组。左心室血压根据超声心动图瓣膜事件和肱动脉收缩压进行无创估算。左心室心肌功参数是通过超声心动图压力-应变环路分析获得的。与对照组相比,FD 组的总体纵向应变(GLS)、总体做功指数、总体做功效率(GWE)、总体建设性做功和总体废物做功均明显较低(P 结论:心肌做功分析可用于诊断心肌梗死:心肌功分析可用于评估 FD 患者的全局和区域心肌功。在本研究中,FD 患者的 GLS 和 GWE 减少,且与 LVH 的存在有关。基底和中部心肌功随左心室功能不全而减少,而心尖心肌功保持不变,这为探索心肌损伤的分布提供了更多价值。
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来源期刊
Orphanet Journal of Rare Diseases
Orphanet Journal of Rare Diseases 医学-医学:研究与实验
CiteScore
6.30
自引率
8.10%
发文量
418
审稿时长
4-8 weeks
期刊介绍: Orphanet Journal of Rare Diseases is an open access, peer-reviewed journal that encompasses all aspects of rare diseases and orphan drugs. The journal publishes high-quality reviews on specific rare diseases. In addition, the journal may consider articles on clinical trial outcome reports, either positive or negative, and articles on public health issues in the field of rare diseases and orphan drugs. The journal does not accept case reports.
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