Two-Dimensional Speckle Tracking Echocardiography in Distinguishing Fabry Disease in Patients with Left Ventricular Hypertrophy
10.3969/j.issn.1005-5185.2025.08.009
- VernacularTitle:二维斑点追踪超声在左心室壁增厚患者中鉴别Fabry病的价值
- Author:
Chonghui WANG
1
;
Han WANG
;
Ying YANG
;
Lin LIU
;
Fangfang FAN
;
Yawen ZHAO
;
Wei ZHANG
;
Yang YU
;
Wei MA
Author Information
1. 北京大学第一医院心内科,北京 100034;北京大学国际医院心内科,北京 100096
- Publication Type:Journal Article
- Keywords:
Fabry disease;
Ultrasonography;
Hypertrophy,left ventricular;
Strain;
Two-dimensional speckle tracking echocardiography;
Diagnosis,differential
- From:
Chinese Journal of Medical Imaging
2025;33(8):848-853,879
- CountryChina
- Language:Chinese
-
Abstract:
Purpose To explore the diagnostic value of two-dimensional speckle tracking echocardiography(2D-STE)in differentiating left ventricular hypertrophy(LVH)caused by Fabry disease from other etiologies.Materials and Methods A total of 23 patients clinically confirmed Fabry disease with LVH(Fabry disease group)in Peking University First Hospital from August 2014 to February 2023,retrospectively.23 patients with hypertensive LVH(hypertensive LVH group)and 23 with hypertrophic cardiomyopathy(HCM)(HCM group)were also included.Conventional echocardiographic parameters and 2D-STE-derived left atrial strain and left ventricular longitudinal strain were analyzed and compared among the three groups.LASSO regression was used to select variables and construct a diagnostic model to differentiate hypertensive LVH,HCM and Fabry disease group.Results The Fabry disease group showed significantly reduced left ventricular global longitudinal strain and segmental left ventricular longitudinal strain in the basal anterior wall,basal anterolateral wall and mid inferior wall compared to the hypertensive LVH group(P<0.05).There were no significant differences in left atrial strain,left ventricular global longitudinal strain and segmental left ventricular longitudinal strain between the Fabry disease and HCM groups(P>0.05).Compared to the conventional echocardiography-based model,the combined model did not significantly improve diagnostic accuracy for differentiating the three etiologies(Z=-1.314--0.594,all P>0.05).Conclusion Compared to patients with hypertensive LVH,HCM and Fabry disease group exhibit varying degrees of decrease in global and segmental longitudinal strain of the left atrium and left ventricle.Additional measurements of left ventricular longitudinal strain and left atrial strain do not provide significant incremental diagnostic value over conventional echocardiography in distinguishing among Fabry disease,hypertensive LVH and HCM.
