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

Francesco De Cobelli, Antonio Esposito, Elena Belloni, Maurizio Pieroni, Gianluca Perseghin, Cristina Chimenti, Andrea Frustaci, Alessandro Del Maschio

Research output: Contribution to journalArticle

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.

Original languageEnglish
JournalAmerican Journal of Roentgenology
Volume192
Issue number3
DOIs
Publication statusPublished - Mar 2009

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Keywords

  • Cardiac MRI
  • Delayed-enhanced MRI
  • Fabry's disease
  • Hypertrophic cardiomyopathy

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging

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