Echocardiographic features of Fabry cardiomyopathy—Comparison with hypertrophy‐matched sarcomeric hypertrophic cardiomyopathy.
Background: The concept of "red flags" has been particularly useful in the etiologic diagnosis of cardiomyopathies such as Fabry disease, as early detection is often essential for treatment response and outcomes. The present study sought to describe the echocardiographic features that may differenti...
| Publicado en: | Echocardiography Vol. 36; no. 11; pp. 2041 - 2050 |
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| Autores principales: | , , , , , |
| Formato: | diagnostic images research tables/charts Journal Article |
| Publicado: |
Wiley-Blackwell
Nov2019
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| Acceso en línea: | Ver este registro en EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=139825216&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 139825216 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 07422822 GSE jtl: Echocardiography issn: 07422822 maglogo: Y pubinfo: dt: Nov2019 vid: 36 iid: 11 pid: 480 pub: Wiley-Blackwell place: Malden, Massachusetts artinfo: ui: 139825216 139825216 139825216 10.1111/echo.14508 139825216 ppf: 2041 ppct: 9 formats: fmt: – @attributes: type: T – @attributes: type: C – @attributes: type: P tig: atl: Echocardiographic features of Fabry cardiomyopathy—Comparison with hypertrophy‐matched sarcomeric hypertrophic cardiomyopathy. aug: au: Militaru, Sebastian Jurcuț, Ruxandra Adam, Robert Roşca, Monica Ginghina, Carmen Popescu, Bogdan A. affil: Department of Cardiology, Expert Center for Rare Genetic Cardiovascular Diseases, Emergency Institute of Cardiovascular Diseases "Prof. Dr. C. C. Iliescu", Bucharest Romania sug: subj: Fabry Disease Ultrasonography Myocardial Diseases Ultrasonography Cardiomyopathy, Hypertrophic Ultrasonography Hypertrophy, Left Ventricular Ultrasonography Echocardiography, Doppler Sensitivity and Specificity Human Prospective Studies Ventricular Ejection Fraction Heart Septum Pathology Age Factors ab: Background: The concept of "red flags" has been particularly useful in the etiologic diagnosis of cardiomyopathies such as Fabry disease, as early detection is often essential for treatment response and outcomes. The present study sought to describe the echocardiographic features that may differentiate Fabry cardiomyopathy from sarcomeric hypertrophic cardiomyopathy (HCM). Methods: Forty patients with left ventricular (LV) hypertrophy were prospectively included and divided into two groups: the Fabry group (20) and the sarcomeric HCM group (20). The two groups were matched for LV hypertrophy (similar maximum wall thickness and indexed LV mass) and age. All patients underwent full echocardiographic evaluation including ventricular strain analysis. Results: The Fabry group had significantly lower LV ejection fraction (63 ± 7 vs 72 ± 7%, P = .001) and higher LV end‐systolic diameter (28 ± 7 vs 22 ± 5 mm, P = .004). LV hypertrophy in Fabry patients was more often concentric, with a significantly lower interventricular septum/posterior wall ratio (1.22 ± 0.63 vs 1.55 ± 0.66, P = .001). Fabry patients had more reduced regional longitudinal strain in the inferolateral part of the LV (−9 ± 5 vs –16 ± 7%), and RV free wall longitudinal strain was also worse in Fabry patients (−23 ± 6 vs –28 ± 5%, P = .027). Conclusion: These parameters are promising echocardiographic features to identify patients with Fabry cardiomyopathy and may help for the detection and subsequent management of these patients. pubtype: Academic Journal doctype: diagnostic images research tables/charts Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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