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Left Ventricular Hemodynamic Forces Changes in Fabry Disease: A Cardiac Magnetic Resonance Study
Jialin Li1, Shichu Liang1, Ziqian Xu2
1Department of Cardiology, West China Hospital, Sichuan University, Chengdu, Sichuan, China.
Insights
Hemodynamic force (HDF) analysis using cardiac MRI reveals increased systolic impulse in Fabry disease (FD) patients, even before left ventricular hypertrophy develops. This finding suggests HDF may serve as an early diagnostic and monitoring tool for FD progression.
Area of Science:
- Cardiology
- Medical Imaging
- Biophysics
Background:
- Hemodynamic force (HDF) derived from cardiac MRI can detect subclinical myocardial dysfunction and early cardiac changes in Fabry disease (FD).
- The specific hemodynamic alterations in FD patients remain incompletely understood.
Purpose of the Study:
- To investigate HDF changes in FD patients.
- To evaluate the potential of HDF measurements as diagnostic markers for early cardiac alterations in FD.
Main Methods:
- A prospective, observational study involving 46 FD patients and 46 healthy controls (HCs).
- Cardiac MRI was performed using 3T, including cine imaging for HDF analysis, late gadolinium enhancement (LGE), and T1 mapping.
- HDF parameters such as systolic impulse and global longitudinal strain were assessed and correlated with disease severity (Mainz Severity Score Index - MSSI).
Main Results:
- FD patients exhibited significantly lower native T1 values and higher systolic impulse compared to HCs.
- Elevated systolic impulse was observed even in the pre-hypertrophic stage of FD.
- Increased myocardial global longitudinal strain and systolic impulse positively correlated with higher MSSI scores.
Conclusions:
- Increased systolic impulse and systolic peak are characteristic hemodynamic changes in FD patients.
- Systolic impulse demonstrates potential as a screening tool for pre-left ventricular hypertrophy (LVH) FD patients.
- Systolic impulse is a promising image-based marker for diagnosing and monitoring disease severity in FD.
Background:
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.
Purpose:
To explore HDF changes in FD and the potential of HDF measurements as diagnostic markers indicating early cardiac changes in FD.
Study Type:
Single-center, prospective, observational study.
Population:
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.
Assessment:
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.
Statistical Tests:
Group comparison tests, logistic regression, and receiver operating characteristic curve (ROC) were performed. A P-value <0.05 was considered statistically significant.
Results:
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).
Data Conclusion:
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.
Plain Language Summary:
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.
Evidence Level:
1 TECHNICAL EFFICACY: Stage 2.
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