In this retrospective, observational study, 45 patients with Anderson-Fabry disease (AFD) underwent CMR with T1 mapping using shortened modified Look-Locker inversion recovery sequences, 2-dimensional echocardiography and quantification of Mainz Severity Score Index (MSSI). LA total strain showed very good correlation with native septal T1, LV maximum wall thickness, atrial volumes, and global or cardiovascular MSSI. A good correlation was found with native T1 values, which in pre-hypertrophic patients with AFD had been shown to provide prognostic information. This study contributes to the characterization of the pre-hypertrophic phenotype of AFD, introducing LA total strain as a potential novel indicator of early cardiac involvement and a possible tool to personalize management decisions in Fabry disease patients.
Atrial Dysfunction Assessed by Cardiac Magnetic Resonance as an Early Marker of Fabry Cardiomyopathy / Bernardini, A.; Camporeale, A.; Pieroni, M.; Pieruzzi, F.; Figliozzi, S.; Lusardi, P.; Spada, M.; Mignani, R.; Burlina, A.; Carubbi, F.; Battaglia, Y.; Graziani, F.; Pica, S.; Tondi, L.; Chow, K.; Boveri, S.; Olivotto, I.; Lombardi, M.. - In: JACC. CARDIOVASCULAR IMAGING. - ISSN 1936-878X. - 13:10(2020), pp. 2262-2264. [10.1016/j.jcmg.2020.05.011]
Atrial Dysfunction Assessed by Cardiac Magnetic Resonance as an Early Marker of Fabry Cardiomyopathy
Carubbi F.Membro del Collaboration Group
;
2020
Abstract
In this retrospective, observational study, 45 patients with Anderson-Fabry disease (AFD) underwent CMR with T1 mapping using shortened modified Look-Locker inversion recovery sequences, 2-dimensional echocardiography and quantification of Mainz Severity Score Index (MSSI). LA total strain showed very good correlation with native septal T1, LV maximum wall thickness, atrial volumes, and global or cardiovascular MSSI. A good correlation was found with native T1 values, which in pre-hypertrophic patients with AFD had been shown to provide prognostic information. This study contributes to the characterization of the pre-hypertrophic phenotype of AFD, introducing LA total strain as a potential novel indicator of early cardiac involvement and a possible tool to personalize management decisions in Fabry disease patients.
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Fabry cardiopathi JACC 2020 j.jcmg.2020.05.011.pdf
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