Layer-specific longitudinal strain in Anderson-Fabry disease at diagnosis: A speckle tracking echocardiography analysis.
Fabry disease
left ventricular hypertrophy
left ventricular strain
left ventricular systolic function
speckle tracking echocardiography
Journal
Echocardiography (Mount Kisco, N.Y.)
ISSN: 1540-8175
Titre abrégé: Echocardiography
Pays: United States
ID NLM: 8511187
Informations de publication
Date de publication:
07 2019
07 2019
Historique:
received:
06
03
2019
revised:
09
05
2019
accepted:
10
05
2019
pubmed:
28
6
2019
medline:
2
10
2020
entrez:
28
6
2019
Statut:
ppublish
Résumé
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. 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 (LSsubendo), subepicardial longitudinal strain (LSsubepi), and strain gradient (LSsubendo-LSsubpepi). 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. LSsubendo was significantly higher than LSsubepi in both groups, but GLS (P < 0.0001), LSsubendo (P = 0.003), and particularly LSsubepi (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 LSsubepi. After adjusting for confounders by multivariate analyses, LV mass index or maximal wall thickness were independently and inversely associated with transmural GLS and LSsubepi, but not with LSsubendo in the AFD group. At receiver operating curve curves, LSsubepi best discriminated AFD and normals. 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.
Sections du résumé
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 (LSsubendo), subepicardial longitudinal strain (LSsubepi), and strain gradient (LSsubendo-LSsubpepi).
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. LSsubendo was significantly higher than LSsubepi in both groups, but GLS (P < 0.0001), LSsubendo (P = 0.003), and particularly LSsubepi (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 LSsubepi. After adjusting for confounders by multivariate analyses, LV mass index or maximal wall thickness were independently and inversely associated with transmural GLS and LSsubepi, but not with LSsubendo in the AFD group. At receiver operating curve curves, LSsubepi 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.
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
1273-1281Informations de copyright
© 2019 Wiley Periodicals, Inc.