{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,14]],"date-time":"2026-03-14T10:52:19Z","timestamp":1773485539086,"version":"3.50.1"},"reference-count":16,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2010,3,5]],"date-time":"2010-03-05T00:00:00Z","timestamp":1267747200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cardiovasc electrophysiol"],"published-print":{"date-parts":[[2010,8]]},"abstract":"<jats:p> <jats:bold>\u03b2\u2010Blockers for LQTS Types 1 and 2.<\/jats:bold>\u2002<jats:italic>Background:<\/jats:italic> Beta\u2010blockers are the mainstay therapy in patients with the congenital long\u2010QT syndrome (LQTS) types 1 and 2. However, limited data exist regarding the efficacy and limitations of this form of medical management within high\u2010risk subsets of these populations.<\/jats:p><jats:p> <jats:italic>Methods and Results:<\/jats:italic> Multivariate analysis was carried out to identify age\u2010related gender\u2010 and genotype\u2010specific risk factors for cardiac events (comprising syncope, aborted cardiac arrest [ACA] or sudden cardiac death [SCD]) from birth through age 40 years among 971 LQT1 (n = 549) and LQT2 (n = 422) patients from the International LQTS Registry. Risk factors for cardiac events included the LQT1 genotype (HR = 1.49, P = 0.003) and male gender (HR = 1.31, P = 0.04) in the 0\u201314 years age group; and the LQT2 genotype (HR = 1.67, P &lt; 0.001) and female gender (HR = 2.58, P &lt; 0.001) in the 15\u201340 years age group. Gender\u2013genotype subset analysis showed enhanced risk among LQT1 males (HR = 1.93, P &lt; 0.001) and LQT2 females (HR = 3.28, P &lt; 0.001) in the 2 respective age groups. Beta\u2010blocker therapy was associated with a significant risk\u2010reduction in high\u2010risk patients, including a 67% reduction (P = 0.02) in LQT1 males and a 71% reduction (P &lt; 0.001) in LQT2 females. Life\u2010threatening events (ACA\/SCD) rarely occurred as a presenting symptom among beta\u2010blocker\u2010treated patients. However, high\u2010risk patients who experienced syncope during beta\u2010blocker therapy had a relatively high rate of subsequent ACA\/SCD (&gt;1 event per 100 patient\u2010years).<\/jats:p><jats:p> <jats:italic>Conclusions:<\/jats:italic> The present findings suggest that beta\u2010blocker therapy should be routinely administered to all high\u2010risk LQT1 and LQT2 patients without contraindications as a first line measure, whereas primary defibrillator therapy should be recommended for those who experience syncope during medical therapy. <jats:italic>(J Cardiovasc Electrophysiol, Vol. 21, pp. 893\u2010901, August 2010)<\/jats:italic><\/jats:p>","DOI":"10.1111\/j.1540-8167.2010.01737.x","type":"journal-article","created":{"date-parts":[[2010,3,5]],"date-time":"2010-03-05T20:10:45Z","timestamp":1267819845000},"page":"893-901","source":"Crossref","is-referenced-by-count":108,"title":["Beta\u2010Blocker Efficacy in High\u2010Risk Patients with the Congenital Long\u2010QT Syndrome Types 1 and 2: Implications for Patient Management"],"prefix":"10.1111","volume":"21","author":[{"given":"ILAN","family":"GOLDENBERG","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"JAMES","family":"BRADLEY","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"ARTHUR","family":"MOSS","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"SCOTT","family":"MCNITT","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"SLAVA","family":"POLONSKY","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"JENNIFER L.","family":"ROBINSON","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"MARK","family":"ANDREWS","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"WOJCIECH","family":"ZAREBA","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"name":"on behalf of the International LQTS Registry 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