Increased Spread of Native T1 Values Assessed With MRI as a Marker of Cardiac Involvement in Fabry Disease.

Abstract

OBJECTIVE. Cardiac involvement is the leading cause of mortality in Fabry disease. Noninvasive markers of cardiac involvement are needed to identify patients at high risk. The purpose of this study was to evaluate the diagnostic potential of segmental native T1 spread as an imaging biomarker in Fabry disease. SUBJECTS AND METHODS. In this prospective study, 43 patients with confirmed Fabry disease (mean ± SD age, 46±14 years; 70% women) and 17 healthy control subjects (mean ± SD age, 44 ±13 years; 53% women) underwent 3-T cardiac MRI including modified Look-Locker inversion recovery T1 mapping. Segmental native T1 spread was calculated as the difference between maximum and minimum segmental native T1 values, expressed as an absolute value and as a relative percentage of global native T1. RESULTS. Absolute and relative segmental native T1 spreads were significantly higher in patients with Fabry disease than in healthy control subjects (absolute median, 115 vs 98 ms [p = 0.004]; relative median, 9.9% vs 8.0% [p < 0.001]) and correlated positively with quantitative late gadolinium enhancement (absolute, r = 0.434, p < 0.001; relative, r = 0.436, p < 0.001), indexed left ventricular mass (absolute, r = 0.316, p = 0.01; relative, r = 0.347, p = 0.007), and global longitudinal strain (absolute, r = 0.289, p = 0.03; relative, r = 0.277, p = 0.03). Relative segmental native T1 spread differentiated patients with Fabry disease from healthy control subjects (odds ratio, 1.44 [95% CI, 1.10-1.89]; p = 0.009). Interob-server agreement was excellent for both absolute (intraclass correlation coefficient, 0.932) and relative (intraclass correlation coefficient, 0.926) segmental native T1 spread. CONCLUSION. Increased native T1 spread is a reproducible imaging biomarker of cardiac involvement in Fabry disease and may be particularly useful in the evaluation of patients who cannot undergo late gadolinium enhancement imaging.