AIMS: To evaluate thoracic aortic dilation in patients with Fabry disease (FD). METHODS AND 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. CONCLUSION: 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, Faculty of Medicine, Lausanne.
AIMS: To evaluate thoracic aortic dilation in patients with Fabry disease (FD). METHODS AND 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. CONCLUSION: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, Faculty of Medicine, Lausanne.
Authors: Sara N Koenig; Stephanie LaHaye; James D Feller; Patrick Rowland; Kan N Hor; Aaron J Trask; Paul Ml Janssen; Freddy Radtke; Brenda Lilly; Vidu Garg Journal: JCI Insight Date: 2017-11-02
Authors: Maria Paola Belfiore; Francesca Iacobellis; Emma Acampora; Martina Caiazza; Marta Rubino; Emanuele Monda; Maria Rosaria Magaldi; Antonietta Tarallo; Marcella Sasso; Valeria De Pasquale; Roberto Grassi; Salvatore Cappabianca; Paolo Calabrò; Simona Fecarotta; Salvatore Esposito; Giovanni Esposito; Antonio Pisani; Luigi Michele Pavone; Giancarlo Parenti; Giuseppe Limongelli Journal: PLoS One Date: 2020-05-19 Impact factor: 3.240
Authors: Roberta Esposito; Ciro Santoro; Giulia Elena Mandoli; Vittoria Cuomo; Regina Sorrentino; Lucia La Mura; Maria Concetta Pastore; Francesco Bandera; Flavio D'Ascenzi; Alessandro Malagoli; Giovanni Benfari; Antonello D'Andrea; Matteo Cameli Journal: J Clin Med Date: 2021-05-06 Impact factor: 4.241