{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,20]],"date-time":"2026-01-20T13:14:47Z","timestamp":1768914887833,"version":"3.49.0"},"reference-count":34,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"3","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["JAHA"],"published-print":{"date-parts":[[2024,2,6]]},"abstract":"<jats:sec sec-type=\"background\" xml:lang=\"en\">\n            <jats:title>Background<\/jats:title>\n            <jats:p xml:lang=\"en\">Sex\u2010specific risk management may improve outcomes in congenital long QT syndrome (LQTS). We recently developed a prediction score for cardiac events (CEs) and life\u2010threatening events (LTEs) in postadolescent women with LQTS. In the present study, we aimed to develop personalized risk estimates for the burden of CEs and LTEs in male adolescents with potassium channel\u2010mediated 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              The prognostic model was derived from the LQTS Registry headquartered in Rochester, NY, comprising 611 LQT1 or LQT2 male adolescents from age 10 through 20\u2009years, using the following variables: genotype\/mutation location, QTc\u2010specific thresholds, history of syncope, and \u03b2\u2010blocker therapy. Anderson\u2010Gill modeling was performed for the end point of CE burden (total number of syncope, aborted cardiac arrest, and appropriate defibrillator shocks). The applicability of the CE prediction model was tested for the end point of the first LTE\u00a0(excluding syncope and adding sudden cardiac death) using Cox modeling. A total of 270 CEs occurred during follow\u2010up. The genotype\u2013phenotype risk prediction model identified low\u2010, intermediate\u2010, and high\u2010risk groups, comprising 74%, 14%, and 12% of the study population, respectively. Compared with the low\u2010risk group, high\u2010risk male subjects experienced a pronounced 5.2\u2010fold increased risk of recurrent CEs (\n              <jats:italic>P<\/jats:italic>\n              &lt;0.001), whereas intermediate\u2010risk patients had a 2.1\u2010fold (\n              <jats:italic>P<\/jats:italic>\n              =0.004) increased risk . At age 20\u2009years, the low\u2010, intermediate\u2010, and high\u2010risk adolescent male patients had on average 0.3, 0.6, and 1.4 CEs per person, respectively. Corresponding 10\u2010year adjusted probabilities for a first LTE were 2%, 6%, and 8%.\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec xml:lang=\"en\">\n            <jats:title>Conclusions<\/jats:title>\n            <jats:p xml:lang=\"en\">Personalized genotype\u2013phenotype risk estimates can be used to guide sex\u2010specific management in male adolescents with potassium channel\u2010mediated LQTS.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1161\/jaha.122.028902","type":"journal-article","created":{"date-parts":[[2024,1,19]],"date-time":"2024-01-19T11:35:00Z","timestamp":1705664100000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":4,"title":["Risk Prediction in Male Adolescents With Congenital Long QT Syndrome: Implications for Sex\u2010Specific Risk Stratification in Potassium Channel\u2010Mediated Long QT Syndrome"],"prefix":"10.1161","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-0468-8511","authenticated-orcid":false,"given":"Milica","family":"Bjelic","sequence":"first","affiliation":[{"name":"Clinical Cardiovascular Research Center, Division of Cardiology University of Rochester Medical Center Rochester NY USA"},{"name":"Department of Anesthesiology St. Elizabeth\u2019s Medical Center Boston University School of Medicine Boston MA USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8717-8298","authenticated-orcid":false,"given":"Ido","family":"Goldenberg","sequence":"additional","affiliation":[{"name":"Clinical Cardiovascular Research Center, Division of Cardiology University of Rochester Medical Center Rochester NY USA"},{"name":"Department of Medicine, Rochester Regional Health Rochester NY USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2485-5025","authenticated-orcid":false,"given":"Arwa","family":"Younis","sequence":"additional","affiliation":[{"name":"Clinical Cardiovascular Research Center, Division of Cardiology University of Rochester Medical Center Rochester NY USA"},{"name":"Department of Cardiovascular Medicine Cleveland Clinic Cleveland OH USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anita Y.","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Biostatistics and Computational Biology University of Rochester Medical Center Rochester NY USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9165-7643","authenticated-orcid":false,"given":"David T.","family":"Huang","sequence":"additional","affiliation":[{"name":"Department of Medicine, Division of Cardiology University of Rochester Medical Center Rochester NY USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ayhan","family":"Yoruk","sequence":"additional","affiliation":[{"name":"Division of Cardiology The University of California, San Francisco Medical Center San Francisco CA USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9003-9384","authenticated-orcid":false,"given":"Mehmet K.","family":"Aktas","sequence":"additional","affiliation":[{"name":"Department of Medicine, Division of Cardiology University of Rochester Medical Center Rochester NY USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9958-1172","authenticated-orcid":false,"given":"Spencer","family":"Rosero","sequence":"additional","affiliation":[{"name":"Department of Medicine, Division of Cardiology University of Rochester Medical Center Rochester NY 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USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1855-1102","authenticated-orcid":false,"given":"Peter J.","family":"Kudenchuk","sequence":"additional","affiliation":[{"name":"Department of Medicine University of Washington Seattle WA USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thomas D.","family":"Rea","sequence":"additional","affiliation":[{"name":"Department of Medicine University of Washington Seattle WA USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8980-8695","authenticated-orcid":false,"given":"Dan E.","family":"Arking","sequence":"additional","affiliation":[{"name":"The McKusick\u2010Nathans Institute, Department of Genetic Medicine John Hopkins University School of Medicine Baltimore MD 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