Characterization of Fabry Disease cardiac involvement according to longitudinal strain, cardiometabolic exercise test, and T1 mapping.
Int J Cardiovasc Imaging. 2020-05-08; 36(7): 1333-1342
DOI: 10.1007/s10554-020-01823-7
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1. Int J Cardiovasc Imaging. 2020 Jul;36(7):1333-1342. doi:
10.1007/s10554-020-01823-7. Epub 2020 May 8.
Characterization of Fabry Disease cardiac involvement according to longitudinal
strain, cardiometabolic exercise test, and T1 mapping.
Réant P(1)(2)(3)(4)(5)(6), Testet E(7)(8), Reynaud A(7)(9), Bourque C(7)(10),
Michaud M(7)(8), Rooryck C(7)(8)(11)(12), Goizet C(7)(8)(12), Lacombe
D(7)(8)(12), de-Précigout V(7), Peyrou J(7), Cochet H(7)(8)(11), Lafitte
S(7)(8)(11)(9).
Author information:
(1)Bordeaux University Hospital, 33000, Bordeaux, France.
.
(2)University of Bordeaux, 33000, Bordeaux, France.
.
(3)IHU Lyric, 33600, Bordeaux-Pessac, France. .
(4)CIC 1401, 33600, Bordeaux-Pessac, France. .
(5)INSERM 1045, Bordeaux, France. .
(6)Hopital Cardiologique Haut-Leveque, Avenue de Magellan, 33604, Pessac, France.
.
(7)Bordeaux University Hospital, 33000, Bordeaux, France.
(8)University of Bordeaux, 33000, Bordeaux, France.
(9)CIC 1401, 33600, Bordeaux-Pessac, France.
(10)Centre Hospitalier Universitaire de Sherbrooke, Sherbrooke, Canada.
(11)IHU Lyric, 33600, Bordeaux-Pessac, France.
(12)INSERM 1211, Bordeaux, France.
In Anderson-Fabry disease (FD), we sought to evaluate relation between left
ventricular (LV) hypertrophy, longitudinal strain (LS), myocardial T1 mapping and
cardiopulmonary exercise parameters, and their prognostic value in term of
cardiovascular outcomes. In this prospective, observational, monocentric study
called “FABRY-Image”, we evaluated consecutive adult FD patients by
echocardiography, cardiac magnetic resonance, and cardiopulmonary exercise
testing. We investigated regional LS, the relations between LV hypertrophy, LS,
T1 mapping, and VO2 peak and VE/VCO2, and the prediction of cardiovascular events
during follow-up. From 2016 to 2019, we included 35 FD patients (44 ± 17 years,
40% male), that were compared with 20 controls. In FD patients, global, basal and
mid-LV LS, as well as mean T1 were significantly altered compared to controls
(p