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.
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.
Authors: Piers C A Barker; Sara K Pasquali; Stephen Darty; Richard J Ing; Jennifer S Li; Raymond J Kim; Stephanie DeArmey; Priya S Kishnani; Michael J Campbell Journal: Mol Genet Metab Date: 2010-07-23 Impact factor: 4.797
Authors: E Di Cesare; F Cademartiri; I Carbone; A Carriero; M Centonze; F De Cobelli; R De Rosa; P Di Renzi; A Esposito; R Faletti; R Fattori; M Francone; A Giovagnoni; L La Grutta; G Ligabue; L Lovato; R Marano; M Midiri; A Romagnoli; V Russo; F Sardanelli; L Natale; J Bogaert; A De Roos Journal: Radiol Med Date: 2012-11-26 Impact factor: 3.469
Authors: François-Pierre Mongeon; Michael Jerosch-Herold; Otávio Rizzi Coelho-Filho; Ron Blankstein; Rodney H Falk; Raymond Y Kwong Journal: JACC Cardiovasc Imaging Date: 2012-09