Key result
Left ventricular hypertrophy was found in 50% of adult patients with Fabry disease undergoing cardiac MRI, and was frequently associated with myocardial fibrosis and increased cardiovascular mortality.
Why the study?
Does cardiac MRI and speckle-tracking echocardiography identify heart involvement in adult patients with Fabry disease?
Observational (n=186)
Yes
Does cardiac MRI and speckle-tracking echocardiography identify heart involvement in adult patients with Fabry disease?
Cardiac involvement is present in about half of adult Fabry disease patients, characterized by left ventricular hypertrophy and fibrosis, and is associated with high mortality, especially in those requiring renal replacement therapy.
May warrant enhanced cardiac monitoring in Fabry disease; leaves open whether MRI-guided intervention alters fibrosis or mortality.
To evaluate the frequency and clinical features of heart involvement in Fabry disease (FD) and diagnostic value of cardiac MRI and speckle-tracking echocardiography.
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Moiseev et al. (2022) conducted an observational in Fabry disease (n=186). Fabry disease was evaluated on Left ventricular hypertrophy on cardiac MRI. Left ventricular hypertrophy was found in 50% of adult patients with Fabry disease undergoing cardiac MRI, and was frequently associated with myocardial fibrosis and increased cardiovascular mortality.
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