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SHR Neuro Krebs Kardio Lipid Stoffw Microb

Mundigler, G; Gaggl, M; Heinze, G; Graf, S; Zehetgruber, M; Lajic, N; Voigtländer, T; Mannhalter, C; Sunder-Plassmann, R; Paschke, E; Fauler, G; Sunder-Plassmann, G.
The endocardial binary appearance ('binary sign') is an unreliable marker for echocardiographic detection of Fabry disease in patients with left ventricular hypertrophy.
Eur J Echocardiogr. 2011; 12(10):744-749 Doi: 10.1093/ejechocard/jer112 [OPEN ACCESS]
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Co-Autor*innen der Med Uni Graz
Fauler Günter
Paschke Eduard
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Abstract:
Aims The binary sign, a binary appearance of the left ventricular endocardial border, was suggested to be an echocardio-graphic hallmark in diagnosing Fabry disease, a hereditary, lysosomal storage disorder. The aim of the present study was to examine the reliability of the binary sign as a screening tool to identify patients with Fabry disease. Methods and results In total 309 subjects with an interventricular septum (IVS) thickness of >= 12 mm were investigated, of which 14 had a confirmed diagnosis of Fabry disease. Urinary globotriaosylceramide testing was used to rule out Fabry disease in the control group. From all patients echocardiographic images of the apical four-chamber view were analysed offline by a blinded observer. A binary sign was seen in 63 patients (20%), 4 had Fabry disease and 59 belonged to the control group. Although the proportion of binary signs in patients with Fabry disease was higher (29%) compared with the control group (20%) this difference was not statistically significant. The sensitivity and specificity were 28% (95% confidence interval (CI): 12-65%) and 80% (95% CI: 76-85%), respectively. In a logistic regression model adjusted for age, sex and presence of Fabry disease, the occurrence of a binary sign was highly dependent on the IVS thickness (odds ratio: 1.21; 95% CI: 1.1-1.35; P < 0.001). Conclusion The endocardial binary appearance is associated with the degree of septal hypertrophy but cannot adequately distinguish between patients with Fabry disease and patients with other causes of left ventricular hypertrophy.
Find related publications in this database (using NLM MeSH Indexing)
Adult -
Aged -
Echocardiography -
Endocardium - ultrasonography
Fabry Disease - complications
Female -
Humans -
Hypertrophy, Left Ventricular - etiology
Male -
Middle Aged -
Reproducibility of Results -

Find related publications in this database (Keywords)
Fabry disease
Binary sign
Left ventricular hypertrophy
Hypertrophic cardiomyopathy
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