{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,3]],"date-time":"2026-09-03T07:33:16Z","timestamp":1788420796804,"version":"build-2803163510"},"reference-count":36,"publisher":"BMJ","issue":"1","license":[{"start":{"date-parts":[[2025,5,27]],"date-time":"2025-05-27T00:00:00Z","timestamp":1748304000000},"content-version":"unspecified","delay-in-days":146,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100002129","name":"Heart Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100002129","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Hartekind Foundation"}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["Open Heart"],"accepted":{"date-parts":[[2025,5,14]]},"published-print":{"date-parts":[[2025,1]]},"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Supravalvular aortic stenosis (SVAS) is a rare condition with limited data on patients beyond childhood. This study aims to investigate the clinical course and outcomes of SVAS in adults.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>All adult (\u226518\u2009years) patients with SVAS, prospectively registered in the Dutch Congenital Cor Vitia database between 2001 and 2019, were included. Survival and event-free survival were assessed. Evolution of peak velocity was analysed using linear mixed models. Differences in previous operated state, sex and Williams-Beuren syndrome were explored.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>65 patients were included (age: 23 (IQR: 20, 31) years, 31% female, 46% previous SVAS correction, 47% Williams-Beuren syndrome). The peak velocity was 2.3\u00b11.0 m\/s at inclusion. Median follow-up time was 13 (IQR: 10, 17) years. Four patients died (one patient after cardiac surgery, two of non-cardiac causes and in one patient the cause of death was unknown) resulting in a 10-year survival of 95% (95% CI 90% to 100%) and event-free survival of 83% (95% CI 74% to 93%). There were no differences in event-free survival between previous operated state (p=0.2), sex (p=0.48) or Williams-Beuren syndrome (p=0.85). 31 cardiovascular events occurred in 15 patients, with the majority being arrhythmias. All SVAS-related interventions (three surgeries in two patients) occurred in unoperated patients (7 (95% CI 2 to 21)\/1000 patient years). Although no patient showed fast progression (\u22650.3\u2009m\/s\/year), the peak velocity evolution over time increased faster in females compared with males (first time spline: 0.8 m\/s, p=0.017).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>In adulthood, SVAS patients showed a stable clinical course without rapid progression. While cardiovascular events occurred in this young cohort, they were mostly obsereved in those with additional congenital heart defects, suggesting a more optimistic view for SVAS itself. No significant differences in outcomes were observed in patients with\/without Williams-Beuren syndrome. Overall, SVAS tends to follow a more benign course in adulthood compared with childhood, potentially allowing for less intensive follow-up- though follow-up should still be individualised based on associated congenital heart defects and cardiovascular risks.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1136\/openhrt-2025-003355","type":"journal-article","created":{"date-parts":[[2025,5,27]],"date-time":"2025-05-27T23:20:18Z","timestamp":1748388018000},"page":"e003355","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":2,"title":["Clinical course and outcomes of supravalvular aortic stenosis in adults"],"prefix":"10.1136","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0009-0007-9903-5324","authenticated-orcid":false,"given":"Adine R","family":"de Keijzer","sequence":"first","affiliation":[{"name":"Department of Cardiology, Erasmus University Medical Centre, Rotterdam, The Netherlands"},{"name":"Department of Cardiothoracic Surgery, Erasmus University Medical Centre, Rotterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zo\u00eb A","family":"Keuning","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Erasmus University Medical Centre, Rotterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3244-8195","authenticated-orcid":false,"given":"Frederike","family":"Meccanici","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Erasmus University Medical Centre, Rotterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roland R J","family":"van Kimmenade","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Radboud University Medical Centre, Nijmegen, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4282-4628","authenticated-orcid":false,"given":"Joost P","family":"van Melle","sequence":"additional","affiliation":[{"name":"Department of Cardiology, University Medical Centre Groningen, Groningen, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1433-9692","authenticated-orcid":false,"given":"Berto J","family":"Bouma","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Amsterdam University Medical Centre, Amsterdam, The Netherlands"},{"name":"European Reference Network for Rare, Low Prevalence and Complex Diseases of the Heart (ERN-GUARD-HEART), Amsterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jolanda","family":"Kluin","sequence":"additional","affiliation":[{"name":"Department of Cardiothoracic Surgery, Erasmus University Medical Centre, Rotterdam, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9132-0418","authenticated-orcid":false,"given":"Monique R M","family":"Jongbloed","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Leiden University Medical Centre, Leiden, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1789-8462","authenticated-orcid":false,"given":"Michiel","family":"Voskuil","sequence":"additional","affiliation":[{"name":"Department of Cardiology, University Medical Centre Utrecht, Utrecht, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6770-3830","authenticated-orcid":false,"given":"Jolien W","family":"Roos-Hesselink","sequence":"additional","affiliation":[{"name":"Department of Cardiology, Erasmus University Medical Centre, Rotterdam, The Netherlands"},{"name":"European Reference Network for Rare, Low 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