Left Ventricular Hemodynamic Forces Changes in Fabry Disease: A Cardiac Magnetic Resonance Study

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.