{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,5]],"date-time":"2025-11-05T14:36:17Z","timestamp":1762353377786,"version":"3.41.2"},"reference-count":32,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"14","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["JAHA"],"published-print":{"date-parts":[[2023,7,18]]},"abstract":"<jats:sec sec-type=\"background\" xml:lang=\"en\">\n            <jats:title>Background<\/jats:title>\n            <jats:p xml:lang=\"en\">An elegant bedside provocation test has been shown to aid the diagnosis of long\u2010QT syndrome (LQTS) in a retrospective cohort by evaluation of QT intervals and T\u2010wave morphology changes resulting from the brief tachycardia provoked by standing. We aimed to prospectively determine the potential diagnostic value of the standing test for LQTS.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec xml:lang=\"en\">\n            <jats:title>Methods and Results<\/jats:title>\n            <jats:p xml:lang=\"en\">\n              In adults suspected for LQTS who had a standing test, the QT interval was assessed manually and automated. In addition, T\u2010wave morphology changes were determined. A total of 167 controls and 131 genetically confirmed patients with LQTS were included. A prolonged heart rate\u2013corrected QT interval (QTc) (men \u2265430\u2009ms, women \u2265450\u2009ms) at baseline before standing yielded a sensitivity of 61% (95% CI, 47\u201374) in men and 54% (95% CI, 42\u201366) in women, with a specificity of 90% (95% CI, 80\u201396) and 89% (95% CI, 81\u201395), respectively. In both men and women, QTc\u2265460\u2009ms after standing increased sensitivity (89% [95% CI, 83\u201394]) but decreased specificity (49% [95% CI, 41\u201357]). Sensitivity further increased (\n              <jats:italic>P<\/jats:italic>\n              &lt;0.01) when a prolonged baseline QTc was accompanied by a QTc\u2265460\u2009ms after standing in both men (93% [95% CI, 84\u201398]) and women (90% [95% CI, 81\u201396]). However, the area under the curve did not improve. T\u2010wave abnormalities after standing did not further increase the sensitivity or the area under the curve significantly.\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec xml:lang=\"en\">\n            <jats:title>Conclusions<\/jats:title>\n            <jats:p xml:lang=\"en\">Despite earlier retrospective studies, a baseline ECG and the standing test in a prospective evaluation displayed a different diagnostic profile for congenital LQTS but no unequivocal synergism or advantage. This suggests that there is markedly reduced penetrance and incomplete expression in genetically confirmed LQTS with retention of repolarization reserve in response to the brief tachycardia provoked by standing.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1161\/jaha.122.026419","type":"journal-article","created":{"date-parts":[[2023,7,8]],"date-time":"2023-07-08T11:21:26Z","timestamp":1688815286000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":5,"title":["Diagnostic Accuracy of the Standing Test in Adults Suspected for Congenital Long\u2010QT Syndrome"],"prefix":"10.1161","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6571-7967","authenticated-orcid":false,"given":"Arja S.","family":"Vink","sequence":"first","affiliation":[{"name":"Department of Clinical and Experimental Cardiology Amsterdam UMC, University of Amsterdam Amsterdam The Netherlands"},{"name":"Department of Pediatric Cardiology Amsterdam UMC, University of Amsterdam, Emma Children\u2019s Hospital Amsterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7780-4427","authenticated-orcid":false,"given":"Ben J. M.","family":"Hermans","sequence":"additional","affiliation":[{"name":"Department of Biomedical Engineering Maastricht University Maastricht The Netherlands"},{"name":"Cardiovascular Research Institute Maastricht (CARIM), Maastricht University Maastricht The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jean\u2010Luc Q.","family":"Hooglugt","sequence":"additional","affiliation":[{"name":"Department of Clinical and Experimental Cardiology Amsterdam UMC, University of Amsterdam Amsterdam The Netherlands"},{"name":"Department of Pediatric Cardiology Amsterdam UMC, University of Amsterdam, Emma Children\u2019s Hospital Amsterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8942-164X","authenticated-orcid":false,"given":"Puck J.","family":"Peltenburg","sequence":"additional","affiliation":[{"name":"Department of Clinical and Experimental Cardiology Amsterdam UMC, University of Amsterdam Amsterdam The Netherlands"},{"name":"Department of Pediatric Cardiology Amsterdam UMC, University of Amsterdam, Emma Children\u2019s Hospital Amsterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2358-0170","authenticated-orcid":false,"given":"Veronique M. F.","family":"Meijborg","sequence":"additional","affiliation":[{"name":"Department of Clinical and Experimental Cardiology Amsterdam UMC, University of Amsterdam Amsterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0629-8541","authenticated-orcid":false,"given":"Nynke","family":"Hofman","sequence":"additional","affiliation":[{"name":"Department of Clinical and Experimental Cardiology Amsterdam UMC, University of Amsterdam Amsterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5276-1475","authenticated-orcid":false,"given":"Sally\u2010Ann B.","family":"Clur","sequence":"additional","affiliation":[{"name":"Department of Pediatric Cardiology Amsterdam UMC, University of Amsterdam, Emma Children\u2019s Hospital Amsterdam The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4823-6974","authenticated-orcid":false,"given":"Nico A.","family":"Blom","sequence":"additional","affiliation":[{"name":"Department of Pediatric Cardiology Amsterdam UMC, University of Amsterdam, Emma Children\u2019s Hospital Amsterdam The Netherlands"},{"name":"Department of Pediatric Cardiology Leiden University Medical Center Leiden The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6897-9700","authenticated-orcid":false,"given":"Tammo","family":"Delhaas","sequence":"additional","affiliation":[{"name":"Department of Biomedical Engineering Maastricht University Maastricht The Netherlands"},{"name":"Cardiovascular Research Institute Maastricht (CARIM), Maastricht University Maastricht The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0528-0852","authenticated-orcid":false,"given":"Arthur A. 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