Abstract
Results Aortic arch PWV was significantly increased in FD patients (6.5±3.1vs 5.0 ±1.5 m/s, p<0.01) Compared to control subjects, patients with FD had also markedly decreased distensibility (2.73±1.14 vs 3.45±1.13 10-2 kPa-1, p<0.01) and increased stiffness index beta (9.4 ±6.7.10-2 vs 5.9±2.7.10-2, p<0.001) in the ascending aorta. Descending aortic stiffness parameters were also impaired with a trend for decreased distensibility (2.26±1.15 vs 3.15 ±1.0 10-2 kPa-1, P=0.06) and significant increased for b-index stiffness (8.5 ±3.9 .10-2 vs 2.9±0.9.10-2, p<0.0001).
Highlights
Aortic thoracic remodelling has been recently described in Fabry disease (FD), no data was available concerning aortic arch stiffness (AAS) in this rare genetic disease
Background: Aortic thoracic remodelling has been recently described in FD, no data was available concerning AAS in this rare genetic disease
Twenty nine males with FD matched with 58 controls for age underwent cardiovascular magnetic resonance imaging (CMR) using cine and phase contrast velocity sequences
Summary
Background: Aortic thoracic remodelling has been recently described in FD, no data was available concerning AAS in this rare genetic disease. Aim of this study was to assess aortic arch stiffness (AAS) parameters in male patients with Fabry disease (FD) using cardiovascular magnetic resonance imaging (CMR)
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
