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  • To evaluate thoracic aortic dilation in patients with Fabry disease (FD). 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. 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. Clinical Trial Registration: Protocol 101/01. Ethics committee,
  • To evaluate thoracic aortic dilation in patients with Fabry disease (FD). 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. 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. Clinical Trial Registration: Protocol 101/01. Ethics committee, (en)
Title
  • Aortic remodelling in Fabry disease
  • Aortic remodelling in Fabry disease (en)
skos:prefLabel
  • Aortic remodelling in Fabry disease
  • Aortic remodelling in Fabry disease (en)
skos:notation
  • RIV/00064165:_____/10:7890!RIV11-MZ0-00064165
http://linked.open...avai/riv/aktivita
http://linked.open...avai/riv/aktivity
  • N
http://linked.open...iv/cisloPeriodika
  • 3
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http://linked.open...aciTvurceVysledku
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  • 247214
http://linked.open...ai/riv/idVysledku
  • RIV/00064165:_____/10:7890
http://linked.open...riv/jazykVysledku
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  • Aorta; Atherosclerosis; Fabry disease; Remodelling; clinical manifestations; aneurysms; recommendations; hypertrophy; dilatation; females; cohort; impact (en)
http://linked.open.../riv/klicoveSlovo
http://linked.open...odStatuVydavatele
  • GB - Spojené království Velké Británie a Severního Irska
http://linked.open...ontrolniKodProRIV
  • [FB1EB9165CC5]
http://linked.open...i/riv/nazevZdroje
  • European Heart Journal
http://linked.open...in/vavai/riv/obor
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http://linked.open...cetTvurcuVysledku
http://linked.open...UplatneniVysledku
http://linked.open...v/svazekPeriodika
  • 31
http://linked.open...iv/tvurceVysledku
  • Linhart, Aleš
  • Paleček, Tomáš
  • Barbey, Frédéric
  • Brakch, Noureddine
  • Eckhardt, Boris
  • Jeanrenaud, Xavier
  • Juli, Christoph
  • Qanadli, Salah D
  • Rizzo, Elena
http://linked.open...ain/vavai/riv/wos
  • 000274338700019
issn
  • 0195-668X
number of pages
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