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Erschienen in:
01.06.2019 | Cardiac Magnetic Resonance (E Nagel and V Puntmann, Section Editors)
Contemporary View of Magnetic Resonance Imaging in Fabry Disease
Erschienen in: Current Cardiovascular Imaging Reports | Ausgabe 6/2019
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Purpose
Fabry disease is a rare lysosomal storage disorder, whose main cause of death is cardiac. CMR provides relevant information that contributes to diagnosis, enabling staging of the disease and an appropriate start of therapy. This review intends to highlight the main strengths of this technique, where a relatively robust scientific evidence supports its use.
Recent Findings
CMR is useful in Fabry disease in a number of aspects. On the one hand, it helps characterize myocardial involvement, enabling accurate chamber size quantification and tissue characterization, by means of LGE, and more recently T1 mapping. Early and appropriate diagnosis of the cardiac disease can guide the start of ERT. Secondly, it has prognostic value, improving risk stratification for device therapy.
Summary
An integrative approach of conventional and new CMR techniques is desirable for early detection of subclinical disease, appropriate disease staging, risk stratification, and therapy guiding. T1 mapping is a particularly promising tool in this field.