{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,10]],"date-time":"2026-04-10T09:18:09Z","timestamp":1775812689645,"version":"3.50.1"},"reference-count":21,"publisher":"Cambridge University Press (CUP)","issue":"5","license":[{"start":{"date-parts":[[2019,5,21]],"date-time":"2019-05-21T00:00:00Z","timestamp":1558396800000},"content-version":"unspecified","delay-in-days":20,"URL":"https:\/\/www.cambridge.org\/core\/terms"},{"start":{"date-parts":[[2019,5,21]],"date-time":"2019-05-21T00:00:00Z","timestamp":1558396800000},"content-version":"unspecified","delay-in-days":20,"URL":"https:\/\/www.cambridge.org\/core\/terms"}],"content-domain":{"domain":["www.cambridge.org"],"crossmark-restriction":true},"short-container-title":["Cardiol Young"],"published-print":{"date-parts":[[2019,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec id=\"S1047951118002299_as1\"><jats:title>Introduction:<\/jats:title><jats:p>This research investigated patients who underwent surgery for a dilated aorta associated with a connective tissue disease or inflammatory vasculitis in children and adolescents.<\/jats:p><\/jats:sec><jats:sec id=\"S1047951118002299_as2\"><jats:title>Materials and Methods:<\/jats:title><jats:p>The medical records of 11 patients who underwent aortic surgery for dilatation resulting from a connective tissue disease or inflammatory vasculitis between 2000 and 2017 were retrospectively reviewed.<\/jats:p><\/jats:sec><jats:sec id=\"S1047951118002299_as3\"><jats:title>Results:<\/jats:title><jats:p>The median age and body weight of the patients were 9.6 years (range 5.4 months\u201315.5 years) and 25.8 kg (range 6.8\u201381.5), respectively. The associated diseases were Marfan syndrome (n = 3), Loeys-Dietz syndrome (n = 3), Kawasaki disease (n = 1), Takayasu arteritis (n = 1), PHACE syndrome (n = 1), tuberous sclerosis (n = 1), and unknown (n = 1). The most common initially affected area was the ascending aorta. During the 66.4 \u00b1 35.9 months of follow-up, two Marfan syndrome patients died, and four patients (one Marfan syndrome and three Loeys-Dietz syndrome) had repeated aortic operation. Except for one patient, the functional class was well maintained in all patients who were followed up.<\/jats:p><\/jats:sec><jats:sec id=\"S1047951118002299_as4\"><jats:title>Conclusion:<\/jats:title><jats:p>Cases of surgical treatment for a dilated aorta associated with a connective tissue disease and inflammatory vasculitis are rare in children and adolescents at our institution. Most of the patients in this study showed a tolerable postoperative course. However, the aorta showed progressive dilation over time even after surgical treatment, especially in patients with Loeys-Dietz syndrome. In these patients, close and more frequent regular follow-up is required.<\/jats:p><\/jats:sec>","DOI":"10.1017\/s1047951118002299","type":"journal-article","created":{"date-parts":[[2019,5,21]],"date-time":"2019-05-21T07:56:46Z","timestamp":1558425406000},"page":"564-569","update-policy":"https:\/\/doi.org\/10.1017\/policypage","source":"Crossref","is-referenced-by-count":1,"title":["Surgery on a dilated aorta associated with a connective tissue disease or inflammatory vasculitis in children and adolescents"],"prefix":"10.1017","volume":"29","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6375-1210","authenticated-orcid":false,"given":"Jae Gun","family":"Kwak","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Woong-Han","family":"Kim","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eung 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