Layer‐specific longitudinal strain in Anderson–Fabry disease?at diagnosis: A speckle tracking echocardiography analysis

摘要

Abstract Background Speckle tracking advancements make?now available the analysis of layer‐specific myocardial deformation. This study investigated multilayer longitudinal strain in Anderson–Fabry disease ( AFD ) patients at diagnosis. Methods In a case–control study, 33 newly diagnosed, untreated AFD patients and 33 healthy age‐ and sex‐matched healthy controls underwent a complete echocardiogram, including assessment of left ventricular ( LV ) transmural global longitudinal strain ( GLS ), subendocardial longitudinal strain ( LS subendo), subepicardial longitudinal strain ( LS subepi), and strain gradient ( LS subendo– LS subpepi). Results Anderson–Fabry disease patients had similar blood pressure, heart rate, and ejection fraction but higher body mass index in comparison with controls. LV mass index, maximal, and relative wall thickness were significantly greater in AFD patients. LS subendo was significantly higher than LS subepi in both groups, but GLS ( P ??0.0001), LS subendo ( P ?=?0.003), and particularly LS subepi (21.4?±?1.7 vs 18.8?±?1.4%, P ??0.0001) were lower in AFD patients than in controls. Accordingly, LS gradient was higher in AFD patients ( P ?=?0.003). Three patients symptomatic for dyspnoea presented a combination of LV hypertrophy and reduced LS subepi. After adjusting for confounders by multivariate analyses, LV mass index or maximal wall thickness were independently and inversely associated with transmural GLS and LS subepi, but not with LS subendo in the AFD group. At receiver operating curve curves, LS subepi best discriminated AFD and normals. Conclusions In newly diagnosed, untreated AFD patients, layer‐specific strain imaging highlights an impairment of LV longitudinal deformation, mainly involving subepicardial strain and causing increase in longitudinal strain myocardial gradient. These findings could be useful for identifying the mechanisms underlying early LV dysfunction in AFD patients.