February 22, 2009American Journal of Roentgenology123 citations
Delayed-Enhanced Cardiac MRI for Differentiation of Fabry's Disease from Symmetric Hypertrophic Cardiomyopathy
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Key Result
Delayed-enhanced cardiac MRI revealed a specific inferolateral basal mesocardial distribution pattern in Fabry's disease, differentiating it from symmetric hypertrophic cardiomyopathy.
Study Design
Type
Observational
Structured PICO
Does delayed-enhanced cardiac MRI differentiate Fabry's disease from symmetric hypertrophic cardiomyopathy?
P
Population
Patients with Fabry's disease affected by symmetric myocardial hypertrophy and patients with symmetric hypertrophic cardiomyopathy
E
Exposure
Delayed-enhanced cardiac MRI
C
Comparator
Patients with symmetric hypertrophic cardiomyopathy
O
Outcome
Myocardial location and distribution patterns of delayed enhancementsurrogate
Delayed-enhanced cardiac MRI can differentiate Fabry's disease from symmetric hypertrophic cardiomyopathy by identifying a specific mesocardial delayed enhancement pattern in the inferolateral basal segments.
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.
Cite This Study
Cobelli et al. (2009) conducted an observational in Fabry's disease and symmetric hypertrophic cardiomyopathy. Delayed-Enhanced Cardiac MRI was evaluated on Myocardial location and distribution patterns of delayed enhancement. Delayed-enhanced cardiac MRI revealed a specific inferolateral basal mesocardial distribution pattern in Fabry's disease, differentiating it from symmetric hypertrophic cardiomyopathy.