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Characteristics of Early Cardiac Involvement in 45 Patients With Fabry Disease Monitored by Ultrasonic Cardiogram
- Journal of Sun Yat-sen University(Medical Sciences) Vol. 45, Issue 4, Pages: 613-621(2024)
- 作者机构：
1.中山大学附属第一医院超声医学科，广东 广州 510080
2.中山大学附属第一医院心血管内科，广东 广州 510080
- 作者简介：
DONG Yugang； E-mail： 332dong@163.com
YAO Fengjuan； E-mail： yaofj@mail.sysu.edu.cn
- 基金信息：
DOI：10.13471/j.cnki.j.sun.yat-sen.univ（med.sci）.20240711.001
CLC： R541Published：20 July 2024，
Received：05 May 2024，
Accepted：25 June 2024
- 稿件说明：
扫 描 看 全 文
李杰,叶敏,范瑞等.超声心动图监测45例法布雷病患者心脏早期受累的特征分析[J].中山大学学报（医学科学版）,2024,45(04):613-621.
LI Jie,YE Min,FAN Rui,et al.Characteristics of Early Cardiac Involvement in 45 Patients With Fabry Disease Monitored by Ultrasonic Cardiogram[J].Journal of Sun Yat-sen University(Medical Sciences),2024,45(04):613-621.
李杰,叶敏,范瑞等.超声心动图监测45例法布雷病患者心脏早期受累的特征分析[J].中山大学学报（医学科学版）,2024,45(04):613-621. DOI： 10.13471/j.cnki.j.sun.yat-sen.univ（med.sci）.20240711.001.
LI Jie,YE Min,FAN Rui,et al.Characteristics of Early Cardiac Involvement in 45 Patients With Fabry Disease Monitored by Ultrasonic Cardiogram[J].Journal of Sun Yat-sen University(Medical Sciences),2024,45(04):613-621. DOI： 10.13471/j.cnki.j.sun.yat-sen.univ（med.sci）.20240711.001.
摘要
目的
2
分析45例临床确诊的法布雷病（AFD）患者的临床特征，同时应用超声心动图二维斑点追踪技术（2D-STE）评估法布雷病患者应变图像，以期探索法布雷患者心脏早期受累在超声心动图（UCG）上的表现。
方法
2
本研究为观察性研究。共纳入确诊为AFD的患者45例，根据UCG测量左室壁有无肥厚，分为左室壁肥厚组（17例，室间隔或左室后壁厚度≥12 mm）和无左室壁肥厚组（28例）。收集患者基线资料，并对所有患者进行常规UCG检查及二维斑点追踪超声心动图分析。应用TomTec工作站分析超声心动图图像，比较两组的临床基线资料、UCG常规参数及心肌应变特征。
结果
2
本研究共纳入45例AFD患者，年龄（32.33±16.11）岁，其中男性27例（60.00%）。左室壁肥厚组17例（37.78%），无左室壁肥厚组28例（62.22%）。45例患者的左室射血分数（LVEF）均正常（＞50%）。与无左室壁肥厚组相比，左室壁肥厚组患者的靶器官受累比例明显增加。与无左室壁肥厚组相比，左室壁肥厚组患者的E/A，平均E/E'明显升高（
P
＜0.05）；左室壁肥厚组的整体、心肌层、心内膜以及各节段的纵向应变（LS），周向应变（CS）及径向应变（RS）的差异均无统计学意义（
P
值均＞0.05）。AFD患者心肌层的整体和各节段LS、CS的绝对值均低于心内膜的对应值（
P
值均＜0.05）。AFD患者的中间段的LS、RS绝对值较基底段和心尖段高（
P
值均＜0.05）。
结论
2
早期收缩功能障碍与左室壁厚度无明显关系。2D-STE心肌应变可以早期监测AFD室壁肌层受累较心内膜严重，而中间段受累较心尖段和基底段轻。
Abstract
Objective
2
To evaluate the changes in cardiac structure and ventricular function in patients with Anderson-Fabry Disease （AFD） by two-dimensional speckle tracking echocardiography （2D-STE） and to explore the characteristics of their early cardiac involvement.
Methods
2
All 45 patients diagnosed with AFD in this observational study underwent routine ultrasonic cardiogram （UCG） examination and 2D-STE. The patients were divided into 2 groups based on UCG measurements： with left ventricular hypertrophy （interventricular septum or posterior left ventricular wall thickness ≥12 mm） and without left ventricular hypertrophy. TomTec software was used to analyze the echocardiographic images， then the baseline data， UCG routine parameters and myocardial strain of the two groups were compared.
Results
2
The study included 27 males （60.0%） and 18 females （40.0%）， with an average age of （32.33±16.11）， 17 cases （37.78%） with left ventricular hypertrophy and 28 cases （62.22%） without left ventricular hypertrophy. All patients had normal left ventricular ejection fraction （LVEF） （
>
50%）. Compared with those without left ventricular hypertrophy， patients with left ventricular hypertrophy had significantly more target organ involvement， significantly higher E/A and average E/E' ratios （
P
<
0.05）. No statistical difference was found in global and segmental longitudinal strain （LS）， circumferential strain （CS） and radial strain （RS） of the endocardium and myocardium between the two groups （all
P
>
0.05）. There were lower absolute values of global and segmental LS and CS in the myocardium than in the endocardium （all
P
<
0.05）， and higher absolute values of LS and RS in the mid segment than in the basal and apical segments （all
P
<
0.05）.
Conclusions
2
There is no significant association between early systolic dysfunction and left ventricular wall thickness. 2D-STE strain can be used to detect AFD in the early stage. Ventricular wall myocardium exhibits more serious involvement than endocardium and mid segment was less involved than the apical and basal segments.
关键词
法布雷病左室壁肥厚二维斑点追踪超声心动图应变左室功能障碍
Keywords
Anderson-Fabry disease （AFD）left ventricular hypertrophytwo-dimensional speckle tracking echocardiography （2D-STE）strainleft ventricular dysfunction
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