Aortic remodelling in Fabry disease.
OBJECTIVE
To evaluate thoracic aortic dilation in patients with Fabry disease (FD).


RESULTS
A cohort of 106 patients with FD (52 males; 54 females) from three European centres were studied. The diameter of the thoracic aorta was assessed at three levels (sinus of Valsalva, ascending aorta, and descending aorta) using echocardiograms and cardiovascular magnetic resonance imaging. Aortic dilation at the sinus of Valsalva was found in 32.7% of males and 5.6% of females; aneurysms were present in 9.6% of males and 1.9% of females. No aortic dilation was observed in the descending aorta. There was no correlation between aortic diameter at the sinus of Valsalva and cardiovascular risk factors.


CONCLUSIONS
Fabry disease should be considered as a cardiovascular disease that affects the heart and arterial vasculature, including the thoracic aorta. Thus, patients with FD should be closely monitored for the presence, and possible progression and complications of aortic dilation.


BACKGROUND
Protocol 101/01. Ethics committee, Faculty of Medicine, Lausanne.
Department of Transplantation, Faculty of Medicine and Biology, University Hospital, Lausanne, Switzerland. frederic.barbey@chuv.ch
Frédéric
F
Barbey
Salah D
SD
Qanadli
Christoph
C
Juli
Noureddine
N
Brakch
Tomás
T
Palacek
Elena
E
Rizzo
Xavier
X
Jeanrenaud
Boris
B
Eckhardt
Ales
A
Linhart
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D017545:Aortic Aneurysm, Thoracic / Q000473:pathology*; D004452:Echocardiography; D000795:Fabry Disease / Q000473:pathology* / Q000503:physiopathology; D005260:Female; D006801:Humans; D018810:Magnetic Resonance Angiography; D008297:Male; D008875:Middle Aged; D012850:Sinus of Valsalva / Q000473:pathology; D055815:Young Adult