Caractérisation de l’atteinte cardiaque de la maladie de Fabry selon le strain longitudinal, les rehaussements tardifs et le T1 mapping : relation avec le statut fonctionnel, et prédiction des évènements cardiovasculaires

Abstract : Background: Anderson-Fabry disease (FD) is a rare X-linked glycolipid storage disease in which cardiac involvement is a leading cause of morbimortality. Neither relation between echocardiographic and cardiovascular magnetic resonance (CMR) innovative parameters, and exercise testing parameters, nor their prognosis value, have been evaluated yet. Aims: we sought to evaluate the relation between cardiac imaging parameters (i.e. left ventricular (LV) mass, longitudinal strain (LS), native T1 and late gadolinium enhancement) and cardiopulmonary exercise testing in FD patients; and cardiovascular outcomes during follow-up. Methods : in this prospective, monocentric study, we evaluated all consecutive FD patients referred in our tertiary center, performing electrocardiogram, holter monitoring, echocardiography, CMR, and cardiopulmonary exercise testing, that we compared with healthy controls. Results: from June 2016 to November 2017, we included 31 FD patients (mean age 42 ± 16 years, 39% male) with an overall mild degree of disease severity, that were compared with 14 matched healthy controls. Thirteen FD patients (42%) had left ventricular hypertrophy (LVH) (wall thickness >11.0 mm). Mean T1 in FD patients was significantly lower than in controls. Regarding functional capacity, a good correlation was observed between VO2 peak and global (r=-0.55), endocardial (-0.53), and epicardial (r=-0.40) LS. VE/VCO2 slope was correlated with maximal LV wall thickness (r=0.48), mean E/E’ ratio (r=0.42), and T1 value (r=-0.57). During a median follow-up of 23 months, 6 patients presented cardiovascular events. In the Cox univariate analyses, global transmural LS and more specifically basal septal LS, basal global and lateral epicardial LS, LV mass index, and anterior T1 value were significantly predictive of CV event (p< 0.05). Conclusions: our study suggests that longitudinal strain and T1 mapping are relevant for prediction of functional status and cardiovascular outcomes in FD patients.
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Master Thesis
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https://dumas.ccsd.cnrs.fr/dumas-02069704
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Submitted on : Friday, March 15, 2019 - 6:33:46 PM
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Émilie Testet. Caractérisation de l’atteinte cardiaque de la maladie de Fabry selon le strain longitudinal, les rehaussements tardifs et le T1 mapping : relation avec le statut fonctionnel, et prédiction des évènements cardiovasculaires. Médecine humaine et pathologie. 2018. ⟨dumas-02069704⟩

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