{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,29]],"date-time":"2025-10-29T19:31:33Z","timestamp":1761766293595,"version":"3.44.0"},"reference-count":0,"publisher":"SMW Supporting Association","issue":"4142","license":[{"start":{"date-parts":[[2017,10,22]],"date-time":"2017-10-22T00:00:00Z","timestamp":1508630400000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Swiss Med Wkly"],"abstract":"<jats:p>BACKGROUND\n          Previous cross-sectional studies reported a high prevalence of ascending aorta dilations\/aneurysms in male adults with Fabry disease, independently of cardiovascular risk factors.\n        \n        \n          AIMS OF THE STUDY\n          To characterise the remodelling of the ascending aorta in classic Fabry disease under long-term enzyme replacement therapy.\n        \n        \n          METHODS\n          Diameter of the ascending aorta was measured with magnetic resonance imaging at the sino-tubular junction (STJ), and proximal (pAsAo), and distal ascending aorta (dAsAo) at baseline, and after 5 and 10 years of enzyme replacement therapy in 15 adult Fabry patients (10 males; 5 females).\n        \n        \n          RESULTS\n          Over a mean follow-up of 9.5 years, the annual expansion rates measured in 10 males with Fabry disease were 0.41 \u00b1 0.16, 0.36 \u00b1 0.25 and 0.41 \u00b1 0.26 mm\/y at the STJ, pAsAo and dAsAo, respectively. Expansion rate at the pAsAo level in male patients was significantly higher than the expected expansion projected from theoretical normal values: 0.36 \u00b1 0.25 vs 0.13 \u00b1 0.05, p = 0.017. In 5 females, the annual expansion rates at the STJ, pAsAo and dAsAo were 0.14 \u00b1 0.11, 0.21 \u00b1 0.18 and 0.26 \u00b1 0.24 mm\/y, respectively. There was no significant difference from the projected normal expansion rate at the level of the pAsAo: 0.21 \u00b1 0.18 vs 0.13 \u00b1 0.04, p = 0.39.\n        \n        \n          CONCLUSION\n          Our data suggest that the remodelling of the ascending aorta is more pronounced in male patients with Fabry disease under long-term enzyme replacement therapy compared with the progression observed in a large population study.<\/jats:p>","DOI":"10.4414\/smw.2017.14517","type":"journal-article","created":{"date-parts":[[2017,11,29]],"date-time":"2017-11-29T02:55:39Z","timestamp":1511924139000},"page":"w14517","source":"Crossref","is-referenced-by-count":2,"title":["Ascending aortic remodelling in Fabry disease after long-term enzyme replacement therapy"],"prefix":"10.57187","volume":"147","author":[{"given":"Pierre","family":"Monney","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Salah D.","family":"Qanadli","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Steven D.","family":"Hajdu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christel","family":"Tran","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Juerg","family":"Schwitter","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Olivier","family":"Dormond","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fr\u00e9d\u00e9ric","family":"Barbey","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"36756","published-online":{"date-parts":[[2017,10,22]]},"container-title":["Swiss Medical Weekly"],"original-title":[],"link":[{"URL":"https:\/\/smw.ch\/index.php\/smw\/article\/download\/2379\/3643","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/smw.ch\/index.php\/smw\/article\/download\/2379\/3644","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/smw.ch\/index.php\/smw\/article\/download\/2379\/3643","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,15]],"date-time":"2025-08-15T07:48:18Z","timestamp":1755244098000},"score":1,"resource":{"primary":{"URL":"https:\/\/smw.ch\/index.php\/smw\/article\/view\/2379"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,10,22]]},"references-count":0,"journal-issue":{"issue":"4142","published-online":{"date-parts":[[2017,10,9]]}},"URL":"https:\/\/doi.org\/10.4414\/smw.2017.14517","relation":{},"ISSN":["1424-3997"],"issn-type":[{"type":"electronic","value":"1424-3997"}],"subject":[],"published":{"date-parts":[[2017,10,22]]}}}