Large artery and left ventricular remodelling in Fabry disease

In the present study, we determined mechanical properties of the carotid and radial arteries in a cohort of 24 hemizygous male patients with biologically proven Fabry disease (FD), compared with 21 age and sex matched normal controls. We further sought for correlations between left ventricle geometry mesured by MRI, and arterial remodelling. Arterial parameters were obtained with a high precision echotracking device, 10 time more precise than concurrent techniques. We found a severe hypertrophy associated with increased distensibility, both for the carotid and the radial artery. Thickening with age was exacerbated in FD patients. Left ventricule remodelling was dependent on hemodynamic load and proportional to arterial hypertrophy. These results show that tissues of the cardiovascular system tend to be oversensitive to trophic signals in patients with Fabry disease.