OBJECTIVE. Fabry's disease may be difficult to differentiate from symmetric hypertrophic cardiomyopathy. Our aim was to compare the myocardial location and distribution patterns of delayed enhancement between patients with Fabry's disease who are affected by symmetric myocardial hypertrophy and patients with symmetric hypertrophic cardiomyopathy in order to identify a specific sign to best differentiate the two diseases. CONCLUSION. Patients with Fabry's disease-related hypertrophy showed left ventricular (LV) delayed enhancement with a typical and consistently found pattern characterized by the involvement of the inferolateral basal or mid basal segments and a mesocardial distribution that spared the subendocardium. This pattern seems to be specific to Fabry's disease; in fact, patients with symmetric hypertrophic cardiomyopathy had variable locations and distributions of delayed enhancement. These observations may contribute to identifying Fabry's disease as a specific cause of symmetric hypertrophy. © American Roentgen Ray Society

De Cobelli, F., Esposito, A., Belloni, E., Pieroni, M., Perseghin, G., Chimenti, C., et al. (2009). Delayed-enhanced cardiac MRI for differentiation of fabry's disease from symmetric hypertrophic cardiomyopathy. AMERICAN JOURNAL OF ROENTGENOLOGY, 192(3), W97-W102 [10.2214/AJR.08.1201].

Delayed-enhanced cardiac MRI for differentiation of fabry's disease from symmetric hypertrophic cardiomyopathy

PERSEGHIN, GIANLUCA;
2009

Abstract

OBJECTIVE. Fabry's disease may be difficult to differentiate from symmetric hypertrophic cardiomyopathy. Our aim was to compare the myocardial location and distribution patterns of delayed enhancement between patients with Fabry's disease who are affected by symmetric myocardial hypertrophy and patients with symmetric hypertrophic cardiomyopathy in order to identify a specific sign to best differentiate the two diseases. CONCLUSION. Patients with Fabry's disease-related hypertrophy showed left ventricular (LV) delayed enhancement with a typical and consistently found pattern characterized by the involvement of the inferolateral basal or mid basal segments and a mesocardial distribution that spared the subendocardium. This pattern seems to be specific to Fabry's disease; in fact, patients with symmetric hypertrophic cardiomyopathy had variable locations and distributions of delayed enhancement. These observations may contribute to identifying Fabry's disease as a specific cause of symmetric hypertrophy. © American Roentgen Ray Society
Articolo in rivista - Articolo scientifico
Cardiac MRI; Delayed-enhanced MRI; Fabry's disease; Hypertrophic cardiomyopathy; Radiology, Nuclear Medicine and Imaging
English
2009
192
3
W97
W102
reserved
De Cobelli, F., Esposito, A., Belloni, E., Pieroni, M., Perseghin, G., Chimenti, C., et al. (2009). Delayed-enhanced cardiac MRI for differentiation of fabry's disease from symmetric hypertrophic cardiomyopathy. AMERICAN JOURNAL OF ROENTGENOLOGY, 192(3), W97-W102 [10.2214/AJR.08.1201].
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/10281/165250
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