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

作者：Han Wang, Ying Yang, Lin Liu, Yawen Zhao, Li Yang, Wei Zhang, Wei Ma · 发表于：Orphanet Journal of Rare Diseases · 年份：2024 · DOI：10.1186/s13023-024-03396-3 · 被引用次数：5 · 研究领域：Lysosomal Storage Disorders Research、Cardiovascular Function and Risk Factors、Cardiomyopathy and Myosin Studies

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...