Li Jialin, Liang Shichu, Xu Ziqian, Wan Ke, Pu Lutong, Wang Jie, Han Yuchi, Chen Yucheng
Department of Cardiology, West China Hospital, Sichuan University, Chengdu, Sichuan, China.
Department of Radiology, West China Hospital, Sichuan University, Chengdu, Sichuan, China.
J Magn Reson Imaging. 2025 Jul;62(1):116-127. doi: 10.1002/jmri.29700. Epub 2025 Jan 22.
Hemodynamic force (HDF) from cardiac MRI can indicate subclinical myocardial dysfunction, and help identify early cardiac changes in patients with Fabry disease (FD). The hemodynamic change in FD patients remains unclear.
To explore HDF changes in FD and the potential of HDF measurements as diagnostic markers indicating early cardiac changes in FD.
Single-center, prospective, observational study.
Forty-six FD patients (age: 38 ± 12, females: 45.65%) and 46 sex- and age-matched healthy controls (HCs).
FIELD STRENGTH/SEQUENCE: 3 T, cardiac MRI including steady-state free precession cine imaging (during multiple breath-holds), phase-sensitive inversion recovery sequence for late gadolinium enhancement (LGE) imaging, and motion-corrected modified Look-Locker inversion recovery sequence for T1 mapping.
Analysis of strains and HDF were performed on the cine imaging. HDF parameters includes apical-basal force, systolic impulse, systolic peak, systolic-diastolic transition, diastolic deceleration, and atrial thrust. Moreover, FD patients were categorized with left ventricular hypertrophy (LVH+) (the maximal wall thickness >12 mm) or without LVH (LVH-). Mainz Severity Score Index (MSSI) score was calculated to measure the progression of FD.
Group comparison tests, logistic regression, and receiver operating characteristic curve (ROC) were performed. A P-value <0.05 was considered statistically significant.
FD patients showed significantly lower native T1 (1161.1 ± 55.4 vs. 1202.8 ± 42.0 msec) and higher systolic impulse (33.8 ± 9.9 vs. 24.8 ± 9.5%). The systolic impulse in HDF analysis increased even in the pre-hypertrophic stage. The increased myocardial global longitudinal strain (r = 0.419) and systolic impulse (r = 0.333) showed positive correlations with a higher MSSI score. The AUC of systolic impulse and global native T1 showed no significant difference (0.764 vs. 0.790, P = 0.784).
Increased systolic impulse and systolic peak can be observed in FD patients. Systolic impulse showed potential ability for screening pre-LVH FD patients and correlated with disease severity in FD patients.
This study explored hemodynamic changes in patients with Fabry disease (FD) using hemodynamic force (HDF) analysis based on cardiac MRI. 46 FD patients were included and analysis of cardiac function, native T1, strains, and hemodynamic changes on cardiac MRI images were performed. The results showed that systolic impulse and systolic peak of HDF analysis were increased in FD patients, and systolic impulse may increase even in the pre-hypertrophic stage. Systolic impulse was correlated with disease severity in patients with FD, which may be a potential image-based diagnosis and monitoring marker in FD patients.
1 TECHNICAL EFFICACY: Stage 2.
心脏磁共振成像（MRI）中的血流动力学力（HDF）可提示亚临床心肌功能障碍，并有助于识别法布里病（FD）患者的早期心脏变化。FD患者的血流动力学变化尚不清楚。
探讨FD患者的HDF变化以及HDF测量作为诊断标志物提示FD患者早期心脏变化的潜力。
单中心、前瞻性、观察性研究。
46例FD患者（年龄：38±12岁，女性占45.65%）和46例年龄及性别匹配的健康对照者（HCs）。
场强/序列：3T，心脏MRI，包括稳态自由进动电影成像（在多次屏气期间）、用于延迟钆增强（LGE）成像的相位敏感反转恢复序列以及用于T1 mapping的运动校正改良Look-Locker反转恢复序列。
对电影成像进行应变和HDF分析。HDF参数包括心尖-心底力、收缩期冲动、收缩期峰值、收缩期-舒张期转换、舒张期减速和心房推力。此外，FD患者分为左心室肥厚（LVH+）（最大壁厚>12mm）或无左心室肥厚（LVH-）。计算美因茨严重程度评分指数（MSSI）以衡量FD的进展。
进行组间比较检验、逻辑回归和受试者工作特征曲线（ROC）分析。P值<0.05被认为具有统计学意义。
FD患者的固有T1显著降低（1161.1±55.4对1202.8±42.0毫秒），收缩期冲动较高（33.8±9.9对24.8±9.5%）。即使在肥厚前期，HDF分析中的收缩期冲动也增加。增加的心肌整体纵向应变（r = ）和收缩期冲动（r = ）与较高的MSSI评分呈正相关。收缩期冲动和整体固有T1的AUC无显著差异（0.764对0.790，P = 0.784）。
FD患者可观察到收缩期冲动和收缩期峰值增加。收缩期冲动显示出筛查LVH前期FD患者的潜在能力，并与FD患者的疾病严重程度相关。
本研究使用基于心脏MRI的血流动力学力（HDF）分析探讨了法布里病（FD）患者的血流动力学变化。纳入46例FD患者，对心脏MRI图像上的心脏功能、固有T1、应变和血流动力学变化进行了分析。结果显示，FD患者HDF分析的收缩期冲动和收缩期峰值增加，甚至在肥厚前期收缩期冲动可能增加。收缩期冲动与FD患者的疾病严重程度相关，这可能是FD患者潜在的基于影像的诊断和监测标志物。
1 技术效能：2级
