Myocardial Fibrosis Quantification Methods by Cardiovascular Magnetic Resonance Imaging in Patients with Fabry...

Area of Science:

• Cardiology
• Medical Imaging
• Genetics

Background:

• Late gadolinium enhancement (LGE) on cardiac magnetic resonance (CMR) indicates cardiac events in Fabry disease (FD).
• Accurate quantification of LGE is essential for risk stratification in FD patients.
• Standardized LGE quantification methods are needed for reproducible clinical assessment.

Purpose of the Study:

• To determine the most reliable and reproducible technique for quantifying LGE in FD patients.
• To compare the performance of various LGE quantification methods, including standard deviations (SD), full width at half maximum (FWHM), visual assessment with threshold (VAT), and manual methods (MM).

Main Methods:

• Twenty FD patients with LGE underwent CMR on a single scanner with identical LGE sequences.
• LGE quantification was performed using grayscale thresholds (2-6 SD above remote myocardium), FWHM, VAT, and MM.
• Intra- and interobserver reproducibility and variability were assessed using intraclass correlation coefficients (ICC) and coefficient of variation (CV).

Main Results:

• Significant variations in LGE quantification were observed across methods (p < 0.0001).
• Visual assessment with threshold (VAT) and 5 SD demonstrated excellent interobserver reliability (ICC 0.94) and low intraobserver variability (6% CV).
• The FWHM method showed the lowest reliability, with high intra- and interobserver variability (63% and 94% CV).

Conclusions:

• Multiple LGE quantification methods show good to excellent reproducibility in FD patients.
• Visual assessment with threshold (VAT) and 5 SD are the most reliable and reproducible techniques for LGE quantification in FD.
• The FWHM method is the least reliable for LGE quantification in this patient cohort, highlighting the impact of technique choice on fibrosis assessment.