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Figure 1 Two-dimensional echocardiography in four-chamber apical view and left ventricular endomyocardial biopsy from two patients (Patient #4 and Patient #18 of Table 2) with Fabry’s disease cardiomyopathy (A,D and B,E, respectively) and a patient with hypertrophic cardiomyopathy (C,F). Comparison of the three echocardiographic frames reveals the presence of a binary appearance of left ventricular endocardial border in the two Fabry patients (A,B). This echocardiographic finding reflects the glycosphingolipids compartmentalization involving a thickened endocardium (End) with enlarged and engulfed smooth muscle cells (SMC), a subendocardial empty space (SES), and a prominent involvement of subendocardial myocardial layer (SL), while the middle layer (ML) appears partially spared (D,E). The echocardiographic pattern is absent in hypertrophic cardiomyopathy (C), despite a similar thickening of the endocardium (F).