Key result
Genotype-positive Fabry disease was associated with a greater left ventricular mass index compared to controls (82 vs 61 g/m2, p=0.001), with CMR detecting cardiac involvement in 48% of patients.
Why the study?
Does cardiovascular magnetic resonance detect early cardiac involvement in genotype-positive Fabry disease patients compared to matched controls?
Case-Control (n=89)
Does cardiovascular magnetic resonance detect early cardiac involvement in genotype-positive Fabry disease patients compared to matched controls?
Absolute Event Rate: 82% vs 61%
p-value: p=0.001
CMR can detect early cardiac involvement, including increased papillary muscle mass and late gadolinium enhancement, in Fabry disease patients even before the development of left ventricular hypertrophy.
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CMR may identify subclinical cardiac involvement in genotype-positive Fabry disease; hypothesis-generating for screening protocols pending prospective validation.
Kozor et al. (2016) conducted a case-control in Fabry disease (n=89). Fabry disease vs. Matched controls was evaluated on Left ventricular mass (LVM) index (p=0.001). Genotype-positive Fabry disease was associated with a greater left ventricular mass index compared to controls (82 vs 61 g/m2, p=0.001), with CMR detecting cardiac involvement in 48% of patients.
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