{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,30]],"date-time":"2026-10-30T16:43:16Z","timestamp":1793378596289,"version":"4.5.0"},"reference-count":32,"publisher":"Oxford University Press (OUP)","issue":"29","license":[{"start":{"date-parts":[[2024,5,16]],"date-time":"2024-05-16T00:00:00Z","timestamp":1715817600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100009703","name":"Fondazione Regionale per la Ricerca Biomedica","doi-asserted-by":"publisher","award":["EJPRD19-187"],"award-info":[{"award-number":["EJPRD19-187"]}],"id":[{"id":"10.13039\/501100009703","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100009703","name":"Fondazione Regionale per la Ricerca Biomedica","doi-asserted-by":"publisher","award":["825575"],"award-info":[{"award-number":["825575"]}],"id":[{"id":"10.13039\/501100009703","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Ricerca Corrente from Italian Ministry of Health"},{"name":"Registro delle Canalopatie Cardiache"},{"name":"Bibliosan","id":[{"id":"https:\/\/ror.org\/020hx9j68","id-type":"ROR","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,8,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background and Aims<\/jats:title>\n                    <jats:p>Risk scores are proposed for genetic arrhythmias. Having proposed in 2010 one such score (M-FACT) for the long QT syndrome (LQTS), this study aims to test whether adherence to its suggestions would be appropriate.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>LQT1\/2\/3 and genotype-negative patients without aborted cardiac arrest (ACA) before diagnosis or cardiac events (CEs) below age 1 were included in the study, focusing on an M-FACT score \u22652 (intermediate\/high risk), either at presentation (static) or during follow-up (dynamic), previously associated with 40% risk of implantable cardioverter defibrillator (ICD) shocks within 4 years.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Overall, 946 patients (26 \u00b1 19 years at diagnosis, 51% female) were included. Beta-blocker (\u03b2B) therapy in 94% of them reduced the rate of those with a QTc \u2265500\u2005ms from 18% to 12% (P &amp;lt; .001). During 7 \u00b1 6 years of follow-up, none died; 4% had CEs, including 0.4% with ACA. A static M-FACT \u22652 was present in 110 patients, of whom 106 received \u03b2Bs. In 49\/106 patients with persistent dynamic M-FACT \u22652, further therapeutic optimization (left cardiac sympathetic denervation in 55%, mexiletine in 31%, and ICD at 27%) resulted in just 7 (14%) patients with CEs (no ACA), with no CEs in the remaining 57. Additionally, 32 patients developed a dynamic M-FACT \u22652 but, after therapeutic optimization, only 3 (9%) had CEs. According to an M-FACT score \u22652, a total of 142 patients should have received an ICD, but only 22\/142 (15%) were implanted, with shocks reported in 3.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Beta-blockers often shorten QTc, thus changing risk scores and ICD indications for primary prevention. Yearly risk reassessment with therapy optimization leads to fewer ICD implants (3%) without increasing life-threatening events.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/eurheartj\/ehae289","type":"journal-article","created":{"date-parts":[[2024,4,26]],"date-time":"2024-04-26T07:50:42Z","timestamp":1714117842000},"page":"2647-2656","source":"Crossref","is-referenced-by-count":30,"title":["Long QT syndrome: importance of reassessing arrhythmic risk after treatment initiation"],"prefix":"10.1093","volume":"45","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2290-3126","authenticated-orcid":false,"given":"Veronica","family":"Dusi","sequence":"first","affiliation":[{"name":"Department of Medical Sciences, University of Turin , Turin , Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1090-3292","authenticated-orcid":false,"given":"Federica","family":"Dagradi","sequence":"additional","affiliation":[{"name":"Center for 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University of Milano-Bicocca , Milano , Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0008-8902-993X","authenticated-orcid":false,"given":"Paolo","family":"Cerea","sequence":"additional","affiliation":[{"name":"Center for Cardiac Arrhythmias of Genetic Origin and Laboratory of Cardiovascular Genetics, Istituto Auxologico Italiano, IRCCS , Via Pier Lombardo, 22, 20135 Milan , Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-3902-153X","authenticated-orcid":false,"given":"Fulvio L F","family":"Giovenzana","sequence":"additional","affiliation":[{"name":"Center for Cardiac Arrhythmias of Genetic Origin and Laboratory of Cardiovascular Genetics, Istituto Auxologico Italiano, IRCCS , Via Pier Lombardo, 22, 20135 Milan , Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5596-1349","authenticated-orcid":false,"given":"Giulia","family":"Musu","sequence":"additional","affiliation":[{"name":"Center for Cardiac Arrhythmias of Genetic Origin and Laboratory of Cardiovascular Genetics, Istituto Auxologico Italiano, IRCCS , Via Pier Lombardo, 22, 20135 Milan , Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0675-4845","authenticated-orcid":false,"given":"Matteo","family":"Pedrazzini","sequence":"additional","affiliation":[{"name":"Center for Cardiac Arrhythmias of Genetic Origin and Laboratory of Cardiovascular Genetics, Istituto Auxologico Italiano, IRCCS , Via Pier Lombardo, 22, 20135 Milan , 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