T1 Mapping with Cardiac MRI Detects Fabry Disease

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Cardiac MRI

Based on a new study, T1 mapping with cardiac MRI was a highly sensitive and particular parameter for the detection of Fabry disease.

“Current imaging evaluations of the heart [for Fabry disease] are suboptimal. The goal of the current study [was] to evaluate the potential of quantitative T1 mapping with cardiovascular MRI as a disease-specific imaging biomarker,” Richard B. Thompson, PhD, from the University of Alberta, Canada, and colleagues wrote.

The study involved 31 patients with Fabry disease, 23 healthy participants and 21 participants with concentric remodeling or hypertrophy. All patients received cardiac MRI so the researchers could calculate and measure left ventricular morphology, function, delayed enhancement, myocardial T1 values and derived parameters.

Based on the results, average noncontrast myocardial T1 values were lower in patients with Fabry disease (1,070 ms) as opposed to controls (1,177 ms) and those with concentric remodeling or hypertrophy (1,207 ms; P.05).

The researchers conducted single-voxel nuclear MR spectroscopy on four patients with Fabry disease and on four healthy patients. Those set of results revealed a negative linear connection between lipid content and noncontrast T1values (r=–0.9; P=.002).

Women in all groups had lower LV mass and wall thickness longer myocardial T1 values and larger extracellular volume as opposed to the men.

“These results illustrate the ability of cardiac MRI coupled with [nuclear] MR spectroscopy to further characterize substrate for cardiomyopathy and serve as an imaging-based biomarker,” Thompson and colleagues wrote.


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