P1532 Segmental assessment of left ventricle in patients with Fabry's disease
| العنوان: | P1532 Segmental assessment of left ventricle in patients with Fabry's disease |
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| المؤلفون: | M S Rodolico, D C Faro, Corrado Tamburino, S Licciardi, Valentina Losi, Ines Monte |
| المصدر: | EUROPEAN HEART JOURNAL-CARDIOVASCULAR IMAGING 21 (2020): i1008–i1008. doi:10.1093/ehjci/jez319.954 info:cnr-pdr/source/autori:Valentina Losi; Margherita Stefania Rodolico; Denise Cristiana Faro; Salvatore Licciardi; Corrado Tamburino; Ines Paola Monte./titolo:P1532 Segmental assessment of left ventricle in patients with Fabry' s disease./doi:10.1093%2Fehjci%2Fjez319.954/rivista:EUROPEAN HEART JOURNAL-CARDIOVASCULAR IMAGING/anno:2020/pagina_da:i1008/pagina_a:i1008/intervallo_pagine:i1008–i1008/volume:21 |
| بيانات النشر: | Oxford University Press (OUP), 2020. |
| سنة النشر: | 2020 |
| مصطلحات موضوعية: | medicine.medical_specialty, business.industry, General Medicine, Anderson-Fabry disease, Fabry's disease, medicine.anatomical_structure, Ventricle, Internal medicine, medicine, Cardiology, Radiology, Nuclear Medicine and imaging, In patient, Cardiology and Cardiovascular Medicine, business |
| الوصف: | Anderson-Fabry disease (AFD) is a rare X-linked metabolic disorder due to deficiency in lysosomal enzyme activity of a-galactosidase A, resulting in pathological accumulation of glycosphingolipids in several tissues and a progressive multi-organ dysfunction. The Global Longitudinal Strain (GLS) of the left ventricle (LV) by Speckle-tracking echocardiography (STE) has been show to detect subclinical cardiac involvement in many cardiomyopathies. The Mechanical Dispersion (MD), derived from STE is considered able to reflect a heterogeneous myocardial contraction, evaluated in many cardiopathies. A reduction in GLS was associated with myocardial fibrosis in the subclinical stages of AFD cardiomyopathy, but there is no MD data in AFD. The aim of the study was to evaluate the distribution of GLS and MD of LV in patients with AFD. 47 consecutive AFD adult patients treated (37F, 12 M, age 45 + 17 years) were examined by a complete echocardiographic examination from a Vivid E95 ultrasound scanner (Horten, Norway). The STE post-processing was performed by Echopac 2.02, in apical long-axis, 4-chambers, and 2-chambers views to determine GLS and MD. Data were expressed as mean± standard deviation. The comparison between the AFD group and 20 normal subjects (N) was performed with unpaired T-test. Compared to N, the AFD group showed higher values of LVMi, LAVi, E/E’, and MD, but a lower value of GLS (Table). GLS was significantly lower in the basal (sept p In patients with AFD, MD added to GLS seems to be a promising tool for the early diagnosis of segmental dysfunction . Table1: Results AFD N p< m SD m SD LVMi g/sqm 77.1 29.8 62.3 14.1 0.008 LVEF % 65.7 5 63.6 3.5 0.04 LAVi ml/sqm 25.5 11 18.2 4.5 0.000 E/E" 8.3 3 5.4 1.1 0.000 TV vmax m/s 2.2 0.4 2.1 0.3 - LV GLS % -17 4 -20 2 0.003 LV MD ms 52 38 29 8 0.000 |
| تدمد: | 2047-2412 2047-2404 |
| DOI: | 10.1093/ehjci/jez319.954 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::d64ff19fd51c95b891e63d28d0ff4a95 https://doi.org/10.1093/ehjci/jez319.954 |
| Rights: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....d64ff19fd51c95b891e63d28d0ff4a95 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 20472412 20472404 |
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| DOI: | 10.1093/ehjci/jez319.954 |