Why the study?
Long-term cardiac involvement in Anderson-Fabry disease leads to complications, and this review aims to explore advanced CMR techniques as diagnostic and prognostic biomarkers.
Advanced CMR techniques provide comprehensive diagnostic and prognostic tissue characterization for evaluating cardiac involvement in Anderson-Fabry disease.
May aid cardiac evaluation in Anderson-Fabry disease; leaves open prospective validation and standardization of these CMR techniques.
Anderson-Fabry disease (AFD) is a rare multisystem X-linked lysosomal storage disorder caused by α-galactosidase A enzyme deficiency. Long-term cardiac involvement in AFD results in left ventricular hypertrophy and myocardial fibrosis, inducing several complications, mainly arrhythmias, valvular dysfunction, and coronary artery disease. Cardiac magnetic resonance (CMR) represents the predominant noninvasive imaging modality for the assessment of cardiac involvement in the AFD, being able to comprehensively assess cardiac regional anatomy, ventricular function as well as to provide tissue characterization. This review aims to explore the role of the most advanced CMR techniques, such as myocardial strain, T1 and T2 mapping, perfusion and hybrid imaging, as diagnostic and prognostic biomarkers.
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Ponsiglione et al. (2023) studied this question.
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